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1
Palmer, K. T. "Raynaud's phenomenon, vibration induced white finger, and difficulties in hearing." Occupational and Environmental Medicine 59, no. 9 (September 1, 2002): 640–42. http://dx.doi.org/10.1136/oem.59.9.640.
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Lau, C. S., A. O'Dowd, and J. J. Belch. "White blood cell activation in Raynaud's phenomenon of systemic sclerosis and vibration induced white finger syndrome." Annals of the Rheumatic Diseases 51, no. 2 (February 1, 1992): 249–52. http://dx.doi.org/10.1136/ard.51.2.249.
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Bovenzi, Massimo. "Digital arterial responsiveness to cold in healthy men, vibration white finger and primary Raynaud's phenomenon." Scandinavian Journal of Work, Environment & Health 19, no. 4 (August 1993): 271–76. http://dx.doi.org/10.5271/sjweh.1474.
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Palmer, Keith T., Michael J. Griffin, Holly Syddall, Cyrus Cooper, and David Coggon. "The clinical grading of Raynaud's phenomenon and vibration-induced white finger: relationship between finger blanching and difficulties in using the upper limb." International Archives of Occupational and Environmental Health 75, no. 1 (January 2002): 29–36. http://dx.doi.org/10.1007/s004200100275.
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Greenstein, D., and R. C. Kester. "Acute Vibration—Its Effect on Digital Blood Flow by Central and Local Mechanisms." Proceedings of the Institution of Mechanical Engineers, Part H: Journal of Engineering in Medicine 206, no. 2 (June 1992): 105–8. http://dx.doi.org/10.1243/pime_proc_1992_206_274_02.
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In order to establish underlying mechanisms responsible for the vasospastic disorder vibration white finger (VWF), the acute effect of vibration on digital blood flow was assessed. Thirteen patients with primary Raynaud's phenomenon (RP), 15 patients with VWF and 13 controls were exposed to acute vibration in the middle digit of one hand for 1 and 3 minutes. Blood flow was measured in that digit and in the middle finger of the contralateral hand using strain gauge plethysmography before and after vibration. The measurements were then repeated following digital nerve blockade using 2% Lignocaine in the vibrated digit. The results demonstrate that vibration affects digital blood flow through two independent mechanisms. Vibration appears to cause both an axonal vasoconstrictor reflex and an active local vasodilation phenomenon. In patients with established vasospastic disorders this vasoconstrictor reflex is exaggerated.
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Jepsen, Jørgen R., and Jane A. Simonsen. "Raynaud's Phenomenon in a Slap Bass Player: A Case Report." Medical Problems of Performing Artists 31, no. 1 (March 1, 2016): 51–53. http://dx.doi.org/10.21091/mppa.2016.1009.
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OBJECTIVE: Secondary Raynaud’s phenomenon is a frequent condition related to occupational exposure to local vibration but has not been described in musicians. This study aims to describe cold-induced blanching of the right second and (in particular) third digits in a 67-year-old double bass player following decades of cumulative repetitive blunt trauma to the fingers from slapping the strings. METHODS: A physical examination was undertaken and systolic blood pressure measured before and after cold provocation. RESULTS: At 10ÅãC the brachial systolic blood pressure was 156 mm Hg while blood pressure was immeasurable at the finger level, corresponding to a finger/brachial index of 0% of the second and third fingers. CONCLUSION: This is the first reported case of objectively verified, playing-related Raynaud’s phenomenon in a musician.
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Merla, A., G. L. Romani, S. Di Luzio, L. Di Donato, G. Farina, M. Proietti, S. Pisarri, and F. Salsano. "Raynaud's Phenomenon: Infrared Functional Imaging Applied to Diagnosis and Drugs Effects." International Journal of Immunopathology and Pharmacology 15, no. 1 (January 2002): 41–52. http://dx.doi.org/10.1177/039463200201500106.
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A non-invasive, innovative approach to the study of Raynaud's Phenomenon is proposed. A group of patients, with respect of a control group, underwent a simultaneous assessment of thermal properties of all ten fingers using infrared functional imaging (IRFI). The assessment highlighted a quite different behaviour between patients with Primary- (PRP) and those with scleroderma - Raynaud's Phenomenon (SSc) and, compared with other existing techniques, seems to be an objective and effective tool to discriminate between PRP and RP secondary to SSc. 18 healthy volunteers (Norm), 20 Primary Raynaud's Phenomenon (PRP) and 20 Secondary Scleroderma (SSc) patients were studied subsequently to clinical evaluation and nail fold capillaroscopy. High-resolution infrared imaging of finger re-warming processes, immediately after a 2 min cold stress, allowed to identify objective parameters. Temperature integral Q (the temperature evaluation of the area under the time-temperature curve along the re-warming period) provided particularly effective figures in describing thermal properties of the fingers. Grand average Q values were (383.4 ± 12.5) °C/min, (502.9 ± 88.1) °C/min and (1022.0 ± 110.2) ±C/min for the PRP, SSc and Normal groups, respectively. Separate evaluation of the temperature integral for each finger leads to very similar results for the fingers of all the PRP patients; a different thermoregulatory response was observed in SSc patients. The sensitivity of the method in order to distinguish healthy from ill fingers was 100 %. The specificity in distinguishing SSc from PRP was 95%. In addition, IRFI parameters provided a better understanding of the impaired control of the finger's temperature in PRP and SSc with respect to the Normal group. This pilot study also applied IRFI for the measurement of drug effects in patients with Raynaud's Phenomenon. Sixteen out of twenty SSc patients were tested in a single 1-hour session of N-acetylcysteine infusion. IRFI clearly documented a significant increase of face and hands temperature during the drug administration. The grand average value of the finger's temperature after the 1 hour NAC administration was (29.6 ± 3.7) °C, while its value before was (27.9 ± 3.7) °C (p<0.001). N-acetylcysteine seems to act as a vasodilator in patients with Raynaud's phenomenon secondary to systemic sclerosis (scleroderma).
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Hirschl, Katzenschlager, Ammer, Melnizky, Rathkolb, and Kundi. "Doppelblinde, randomisierte, placebokontrollierte Low-Level-Laser Therapiestudie bei Patienten mit primärem Raynaudphänomen." Vasa 31, no. 2 (May 1, 2002): 91–94. http://dx.doi.org/10.1024/0301-1526.31.2.91.
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Background: No causal treatment of primary Raynaud’s phenomenon is available due to its unclear aetiology. Low level laser therapy (LLLT) is applied in a multitude of medical conditions often without sufficient evidence of efficacy and established mechanisms. To asses the effect of this therapy in patients with primary Raynaud’s phenomenon a randomised, double blind, placebo controlled cross over study was designed. Patients and methods: Absolute and relative frequency and intensity of vasospastic attacks during three weeks of either LLLT or placebo therapy and results of infrared thermography before onset and at the end of both therapy sequences were evaluated in 15 patients with primary Raynaud’s phenomenon. Results: Frequency of Raynaud’s attacks was not significantly affected by low level laser therapy. Compared to placebo a significantly lower intensity of attacks during laser irradiation was observed, but no transfer effect occurred. Additionally the mean temperature gradient after cold exposure was reduced after laser irradiation, while the number of fingers showing prolonged rewarming was unaffected. Conclusion: Though further studies are necessary to confirm these results we could demonstrate for the first time in a double blind placebo controlled clinical trial that low laser therapy is a potential candidate for an effective therapy of Raynaud`s phenomenon, although effects seem to be of short duration.
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Gaisin, I., Z. Bagautdinova, R. Valeeva, N. Maximov, O. Desinova, R. Shayakhmetova, I. Sabelnikova, et al. "AB0576 INCIDENCE AND CLINICAL MANIFESTATIONS OF RAYNAUD’S PHENOMENON IN RHEUMATIC DISEASES." Annals of the Rheumatic Diseases 79, Suppl 1 (June 2020): 1584.1–1585. http://dx.doi.org/10.1136/annrheumdis-2020-eular.1930.
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Background:Systemic sclerosis (SSc) is a connective tissue disease (CTD) most frequently associated with Raynaud’s phenomenon – RP (96%), followed by mixed CTD (MCTD) (86%), systemic lupus erythematosus – SLE (31%), undifferentiated CTD (30%), rheumatoid arthritis – RA (22%) and Sjogren’s disease – SD (13%)1. RP can manifest as a classical triple-colour change with pallor (ischaemic phase) followed by cyanosis (deoxygenation) and erythema (reperfusion)2,1. However, this triple-colour change only occurs in 19% of cases2,3. Majority of patients report an episodic double-colour change, consisting of pallor and cyanosis, pallor and erythema or cyanosis and erythema2. In a 4.8-year follow-up, 37.2% of RP patients developed rheumatic diseases (RD), 8.1% had other causes, in 54,7% RP remained primary4.Objectives:To study the incidence and manifestations of secondary RP in RD.Methods:A questionnaire survey conducted in 230 patients with RD.Results:RP was detected in 45.6% of RD patients (n=105), 54.4% of patients with RD had no RP (n=125). RP was 4 times more frequent in females than in males (F:M 4:1). In RP group, 87 patients (82%) had autoimmune RD: SSc (55.2%), SLE (17.1%), RA (6%),dermatomyositis (3.8%), cross syndrome (3.8%), MCTD (1.9%), SD (0.9%).Only 84% of RP patients had positive answers to all three questions that characterizeRP (1. Is there an unusual sensitivity of fingers to cold? 2. Do fingers change colorwhen exposed to cold? 3. Do they turn white and/or bluish?). Biphasic color changes (whitening-blueness; whitening-redness; blue-redness) were observed in 33 (31.4%) patients with RP, three-phase changes – in 32 patients (30.5%). Blueness of fingers to cold was more frequent in SLE than in SSc (p=0.027).Redness of fingers to cold occurred more often in cross syndrome, MCTD, SD, RA, vasculitis than in SSc (p<0.001) and in vasculitis than in SLE (p=0.035). In SSc patients, whitening of fingers to cold was more common than redness (p=0.037) and two-/three-phase changes of fingers color in the cold were more frequent than single-phase changes (p<0.001).The frequency of RP attacks was detected more than once a day in 44 (42%) patients. In 73% of cases, RP did not show signs of deep digital ischemia. Digital ulcers (active) were observed in 13 (12.3%) patients, fractures in a finger area – 23 (21.9%), digital scars – 15 (14.2%), phalange amputations – 7 (6.6%).Conclusion:Patients with RD and secondary RP most often have SSC (55%), less often – SLE (17%), RA (6%), DM (3%). In SSc and SLE patients, Raynaud’s reddening of fingers to cold is less common than in other RD. In SSc, two-/three-phase changes of fingers color in the cold are more frequent than single-phase changes. In SLE, fingers turn blue in the cold more often than in SSc.References:[1]Prete M, Fatone MC, Favoino E, Perosa F. Raynaud’s phenomenon: from molecular pathogenesis to therapy.Autoimmun Rev2014;13:655–67.[2]Linnemann B, Erbe M. Raynaud’s phenomenon – assessment and differential diagnoses.Vasa2015;44:166–77.[3]Heidrich H, Helmis J, Fahrig C, Hovelmann R, Martini N. Clinical characteristics of primary, secondary and suspected secondary Raynaud’s syndrome and diagnostic transition in the long-term follow-up. A retrospective study in 900 patients.Vasa2008;37 (Suppl. 73):3–25.[4]Pavlov–Dolijanovic S, Damjanov NS, VujasinovicStupar NZ, Radunovic GL, Stojanovic RM, Babic D. Late appearance and exacerbation of primary Raynaud’s phenomenon attacks can predict future development of connective tissue disease: a retrospective chart review of 3035 patients.RheumatolInt2013;33:921–6.Acknowledgments:Professor LP. Anan’eva, Professor RT. AlekperovDisclosure of Interests:Ilshat Gaisin Speakers bureau: Boehringer Ingelheim, KRKA, Berlin-Chemie Menarini, Sanofi, Zukhra Bagautdinova: None declared, Rosa Valeeva: None declared, Nikolay Maximov Speakers bureau: Pfizer, KRKA, Oxana Desinova: None declared, Rushana Shayakhmetova: None declared, Irina Sabelnikova: None declared, Anna Tukmacheva: None declared, Larisa Gibadullina: None declared, Natalya Burlaeva: None declared, Elena Agareva: None declared, Yulia Ochkurova: None declared, Tatyana Bragina: None declared, Ksenia Alexandrova: None declared, Elvira Reutova: None declared
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Ferrusquia-Toriz, Diana, Cristina Hernández-Díaz, Luis M. Amezcua-Guerra, Lucio Ventura-Ríos, Violeta Higuera-Ortiz, Ana C. Lozada-Navarro, and Luis H. Silveira. "Ultrasound characterization of the nail bed in patients with systemic lupus erythematosus." Lupus 30, no. 4 (January 20, 2021): 608–14. http://dx.doi.org/10.1177/0961203320988609.
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Objective To characterize the ultrasound findings of the nail plate and nail bed in systemic lupus erythematosus (SLE) and its association with nail dystrophy. Methods Thirty-two SLE patients, 36 patients with osteoarthritis (OA) and 20 healthy individuals were studied. High-frequency linear ultrasound was performed in nails of the second to fifth fingers in all participants. Disease activity (SLEDAI-2K index), accrued organ damage (SLICC/ACR index), autoantibody profile, and Raynaud’s phenomenon were also assessed in SLE patients. Results Nail bed thickness in SLE patients was higher than in healthy individuals (1.25 ± 0.31 mm vs 1.17 ± 0.29 mm; P = 0.01) but lower than in OA (1.39 ± 0.37 mm; P < 0.001), while nail plate thickness was similar among groups. Nail dystrophy was found more frequently in SLE and OA than in healthy individuals. SLE patients with nail dystrophy were older than their counterparts with no dystrophy (39.4 ± 10.4 years vs 27.8 ± 5.6 years; P = 0.004), although nail dystrophy showed no association with SLICC/ACR, SLEDAI-2K, nail bed vascularity, or autoantibodies. Conclusions Nail bed in SLE patients is thicker than in healthy individuals but thinner than in OA patients. Nail dystrophy in SLE is associated with advanced age, but not with accrued organ damage, disease activity, Raynaud's phenomenon, or DIP synovitis assessed by ultrasound.
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Beynon, Celia, Gwenan Huws, and Tom Lawson. "Myeloproliferative Disease: An Unusual Cause of Raynaud’s Phenomenon and Digital Ischaemia." Case Reports in Medicine 2016 (2016): 1–2. http://dx.doi.org/10.1155/2016/9675171.
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We describe a 59-year-old female who presented with ischaemic digits, preceded by a 6-month history of Raynaud’s phenomenon affecting her fingers and toes. There were no clinical or laboratory features of primary vasculitis or connective tissue disease, Doppler imaging was normal, and bloods were unremarkable aside from a platelet count of 786 × 109/L (150–400) and white cells of 16 × 109/L (4–11). In view of the thrombocytosis a JAK2 mutation assay was requested which confirmed a JAK2 V617F mutation, suggesting essential thrombocytosis (ET) as the cause. She received treatment with hydroxycarbamide which normalised her platelet count and led to a complete resolution of her Raynaud’s symptoms. Raynaud’s phenomenon is a rare manifestation of ET. Myeloproliferative disorders such as ET should be considered in the differential diagnosis of Raynaud’s phenomenon and vasculitis.
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Roustit, Matthieu, Sophie Blaise, Claire Millet, and Jean-Luc Cracowski. "Impaired transient vasodilation and increased vasoconstriction to digital local cooling in primary Raynaud's phenomenon." American Journal of Physiology-Heart and Circulatory Physiology 301, no. 2 (August 2011): H324—H330. http://dx.doi.org/10.1152/ajpheart.00246.2011.
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Raynaud's phenomenon (RP) is defined as episodic ischemia of the extremities in response to cold. Although the structure of skin capillaries is normal in primary RP, some data suggest impairment of microvascular function. We aimed at testing whether digital skin blood flow was lower in RP than in controls while cooling locally. We further evaluated the contribution of sensory nerves in the response. We recruited 21 patients with primary RP and 20 healthy volunteers matched on age and gender. After a 10-min baseline at 33°C, skin temperature was cooled at 15 or 24°C during 30 min on the forearm and the finger while monitoring perfusion with a custom-design laser Doppler flowmetry probe. Perfusion was also assessed after topical anesthesia. Blood flow was expressed as cutaneous vascular conductance (CVC). Data were subsequently expressed as area above the curve (AAC0–30) of the percentage decrease from baseline CVC (%BL). CVC on the dorsum of the finger was lower in RP patients compared with controls at 15°C (AAC0–30 were 106,237.2 and 69,544.3%BL·s, respectively; P = 0.02) and at 24°C (AAC0–30 were 86,915 and 57,598%BL·s, respectively; P = 0.04) whereas we observed no significant difference on the finger pad and the forearm. Topical anesthesia increased CVC in patients with RP ( P = 0.05), whereas it did not affect reactivity in controls ( P = 0.86). Our study shows exaggerated skin microvascular vasoconstriction to local cooling on the dorsum of the finger in primary RP compared with controls. Part of this abnormal response in primary RP depends on sensitive nerves.
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Heidrich, Helmis, Fahrig, Hövelmann, and Martini. "Clinical characteristics of primary, secondary and suspected secondary Raynaud’s syndrome and diagnostic transition in the long-term follow-up. A retrospective study in 900 patients." Vasa 37, Supplement 73 (November 1, 2008): 3–25. http://dx.doi.org/10.1024/0301-1526.37.s73.3.
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Background: There have so far been only partial studies or studies in fairly small groups of Raynaud’s patients to systematically analyse the clinical features, the beginning of first Raynaud’s attacks, the interval between first Raynaud’s attacks and the first clinical examination, the duration of Raynaud’s attacks, inducing factors, frequency of attacks, localization, coloration, accompanying symptoms, laboratory parameters, instrument-based examinational findings on the first examination, fitness for work, and development of the diagnostic classification in the long term. Such studies are, however, necessary for reasons of differential diagnostics and therapeutics. Patients and methods: The disease data of 900 patients with established Raynaud’s symptoms, who underwent an examination between 1973 and 2002, were at first analysed in retrospect on the basis of their case history. This was followed, in 2002 to 2003, by a mail inquiry of all 900 patients, who were sent a comprehensive questionnaire to confirm history data and supplement clinical findings. Answers were received from 419 patients. In addition, 177 of the 900 patients underwent at least one follow-up examination after 2.8 years (1 month – 18 years), on the average, which allowed checking whether and how the diagnostic classification into primary, secondary and suspected secondary Raynaud’s syndrome had changed. All data were analysed statistically. Results: Of the 900 patients, 77% were women and 23% were men. The mean age on the first examination was 45.1 ± 15.5 years. Calculated on all patients, the first Raynaud’s attacks occurred at the age of 39.5 ± 16.9 years. The beginning of Raynaud’s attacks fell mostly between the 21st and 60th year of life (minimum 3rd year, maximum 88th year). There was no statistical difference between primary, secondary and suspected secondary Raynaud’s syndromes as to the time, ranging from 38.8 to 44.3 years of age, when the symptoms began. The duration of Raynaud’s attacks averaged 23.6 ± 23.5 min.; they occurred every day in 59.1%, once to three times a day in 26.8%, and more than three times a day in 31.1% of the cases. Factors inducing Raynaud’s attacks were cold in 96.8% and stress in 19.2%. Raynaud’s phenomenon was observed in both hands in 90.9% and in one hand only in 8.6% of the cases. A white discoloration only was observed in 39.0%, a white-blue discoloration sequence, in 19.3%, a purely blue discoloration, in 7.6%, and a triphasic colour response, in 19.1%. The latter sign was significantly more frequent in women and secondary Raynaud’s syndrome than in men and primary Raynaud’s syndrome. At 64.6%, secondary Raynaud’s syndrome was significantly more often associated with pain than primary Raynaud’s syndrome (47.9%). The mean systolic blood pressure was 125.6 ± 20.9 mmHg, the mean diastolic pressure, 78.3 ± 10.6 mmHg. Concomitant diseases were equally frequent in primary, secondary and suspected secondary Raynaud’s syndrome. Finger, hand and arm artery occlusions were equally often seen in all three forms of Raynaud’s syndrome. The results of the first examination showed that 72.9% of the 900 patients suffered from primary, 5.3% from secondary, and 21.3% from suspected secondary Raynaud’s syndrome. In the long term, primary and suspected secondary Raynaud’s syndromes showed a diagnostic transition in 19.2% of the cases after 2.8 years, on the average, while secondary Raynaud’s syndromes did not. The clinical symptoms of the groups, as found on the first examination, were not altered by this transition, though. Conclusion: The clinical characteristics of Raynaud’s syndromes (age when first Raynaud’s attacks are observed, inducing factors of Raynaud’s attacks, duration frequency of attacks, localization, coloration, and accompanying symptoms of an attack) are not specific and therefore not suitable to distinguish between primary and secondary Raynaud’s syndrome. The case history, a general clinical examination, immunoserologic and capillaroscopic findings, and repeated examinations during 5 or more years are necessary to differentiate between primary, secondary and suspected secondary Raynaud’s syndromes.
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Ringqvist, Åsa, Tommy Jonason, Jerzy Leppert, and Ivar Ringqvist. "Non-Invasive Investigation of Endothelium-Dependent Dilatation of the Brachial Artery in Women with Primary Raynaud's Phenomenon." Clinical Science 94, no. 3 (March 1, 1998): 239–43. http://dx.doi.org/10.1042/cs0940239.
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1. Primary Raynaud's phenomenon is characterized by white fingers and toes with impaired perfusion in response to cold or emotional stress. The aetiology has not been clarified. In previous studies we have demonstrated a season-linked inability in women with primary Raynaud's phenomenon to raise their plasma cGMP levels in response to whole-body cooling, suggesting a dysfunction of the l-arginine—NO—cGMP pathway. To further elucidate the possibility of such a defect in patients with primary Raynaud's phenomenon, we determined flow-mediated dilatation of the brachial artery. 2. Twenty-two premenopausal, non-smoking women with primary Raynaud's phenomenon (mean age 39 ± 8 years) and 23 healthy controls (mean age 41 ± 7 years) were studied during two winter weeks. The diameter of the right arm brachial artery was measured by high resolution ultrasonography, at rest and during reactive hyperaemia. The investigation was conducted both with the participants at rest at room temperature and after 40 min of whole-body cooling. 3. Both study groups showed a marked attenuation of flow-mediated dilatation during whole-body cooling, which could partly but not solely be explained by a decreased shear rate. There was, however, no significant difference in flow-mediated diameter (D) increase (% flow-mediated dilatation; ΔD/D × 100) between primary Raynaud's phenomenon and controls, either at room temperature (7.8 ± 0.8 and 9.0 ± 0.8) or in response to whole-body cooling (3.8 ± 1.2 and 4.4 ± 0.7). 4. Thus, whole-body cooling markedly impairs flow-mediated dilatation in women. Flow-mediated dilatation is, however, not decreased in women with primary Raynaud's phenomenon at room temperature or during whole-body cooling, indicating that this particular aspect of endothelial function is not impaired in this setting.
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Donoyama, Nozomi, and Norio Ohkoshi. "Electroacupuncture Therapy for Arthralgia and Raynaud's Phenomenon in a Patient with Systemic Lupus Erythematosus." Acupuncture in Medicine 28, no. 1 (March 2010): 49–51. http://dx.doi.org/10.1136/aim.2009.001529.
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A 45-year-old woman with systemic lupus erythematosus presented with multiple arthralgia, coldness in fingers and toes, and Raynaud's phenomenon. Electroacupuncture (EA) therapy was performed in two courses (14 treatment sessions) 1 month apart. A needle was inserted in the proximal (or medial) side of the painful joint and another needle was inserted in the distal (or lateral) side of the same joint and a 50 Hz stimulus was applied (3 s bursts with 1 s gaps) for 15 min. A visual analogue scale was used to evaluate pain intensity. Cold provocation testing was conducted before and after EA sessions to determine the vasomotor response. Visual analogue scale scores were lower after EA sessions than before. Before starting EA, the skin temperature of the right mid fingertip was 27.9°C and that of the left mid fingertip was 28.3°C. In contrast, after the EA sessions, the skin temperature of the right mid fingertip was 34.8°C and that of the left mid fingertip was 34.7°C. In the last EA session, the patient reported that the cold in her fingers and toes had eased and Raynaud's phenomenon, in which nail colour tone changed from white to red, had disappeared. In the cold-provocation test, before EA, the temperature recovery rates of mid fingertips after cold exposure reached over 80% in 20 min. In contrast, after EA had been completed, the temperature recovery rate exceeded 80% in 10 min, thus the delay of temperature recovery was alleviated.
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Ziegler, Sophie, Carina Z�ch, Michael Gschwandtner, Gerald Eckhardt, Ursula Windberger, Erich Minar, Hugo R�diger, and Wolf Osterode. "Thermoregulation and rheological properties of blood in primary Raynaud?s phenomenon and the vibration-induced white-finger syndrome." International Archives of Occupational and Environmental Health 78, no. 3 (March 15, 2005): 218–22. http://dx.doi.org/10.1007/s00420-004-0601-7.
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Pelmear, P. L., R. Kusiak, and D. Leong. "Hand-Arm Vibration Syndrome Associated with Impact Vibration." Journal of Low Frequency Noise, Vibration and Active Control 14, no. 2 (June 1995): 73–79. http://dx.doi.org/10.1177/026309239501400202.
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The data from 141 hand-arm vibration exposed workers examined for hand-arm vibration syndrome (HAVS) was analyzed in a Cox regression model to determine whether smoking, alcohol, the use of gloves, duration of weighted vibration exposure, and vibration impulsiveness affected the rate of onset of Raynaud's phenomenon. The results show that workers with HAVS were more likely to be smokers. No association with alcohol was detected, but none of the men were heavy drinkers. While weighted acceleration levels affected the latent interval for finger blanching, the strongest association was with impulsiveness from high-level impact pneumatic tools (identified by a 20 dB or more difference between the weighted Leq component and the corresponding weighted peak acceleration in the dominant direction). This association had only been recognised previously in pedestal grinders, so its recognition in multiple hand tools has important implications for prevention methodology development and standard setting. The use of gloves was found to reduce the risk from high-level impact pneumatic tools.
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Heidrich. "Functional vascular diseases: Raynauds syndrome, acrocyanosis and erythromelalgia." Vasa 39, no. 1 (February 1, 2010): 33–41. http://dx.doi.org/10.1024/0301-1526/a000003.
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Raynauds syndrome, acrocyanosis and erythromelalgia are functional vascular diseases that differ with respect to prevalence, clinical picture, therapy, prognosis, and impairment of quality of life. Raynauds syndrome occurs in 5 to 20 % of the population in Europe, is observed four times more often in women than in men and appears first at the age of 40 (3 to 80), on the average. Raynauds attacks are characterized by a paroxysmal white-blue-red or just white and blue discoloration of the fingers and toes; the attacks are induced by cold or stress, usually, cease after no more than some minutes (average 23 min.), but can also persist for hours. A distinction must be made between primary (aetiology unknown), secondary (aetiology known) and suspected secondary Raynauds syndromes (causal underlying disease suspected). There are several different therapy options, but not all of them have been substantiated by evidence. Acrocyanosis is rarer than Raynauds syndrome, and contrary to the latter, is characterized by nonparoxysmal, in most cases persistent, painless bluish-red symmetrical discolorations of the hands, feet and knees. It is more frequent in women than in men and becomes manifest before the 25th year of age, on the average (15th to 70th year of age). A distinction is made between primary acrocyanosis without detectable underlying disease and secondary acrocyanosis with a specific underlying disease. No effective therapy for primary acrocyanosis is known, but secondary forms can sometimes be treated. Patients with primary and secondary erythromelalgia, a very rare condition, sustain paroxysmal burning pain with marked reddening of the legs, feet and less often the hands. The attacks are triggered by warmth. Women are affected more often than men. The age of first manifestation is 40 to 55 years, but the first attacks may just as well occur during childhood. There are different therapeutic approaches with occasional success, but no general recommendations.
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Khalife, Jane, Clinton G. Lauritsen, John Liang, and Syed O. Shah. "DHE-Induced Peripheral Arterial Vasospasm in Primary Raynaud Phenomenon: Case Report." Neurohospitalist 9, no. 2 (September 11, 2018): 113–15. http://dx.doi.org/10.1177/1941874418797764.
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Dihydroergotamine (DHE) is primarily a serotonin 5HT1B and 5HT1D receptor agonist used for acute migraine treatment. It is associated with acute vasoconstriction mediated through the 5HT1B receptor and is contraindicated in patients with history of cardiac disease and peripheral vascular disease. We present a case of acute peripheral arterial vasospasm in a patient with primary Raynaud phenomenon while receiving inpatient treatment for status migrainosus with intravenous (IV) DHE. The patient is a 35-year-old female with a history of chronic migraine and primary Raynaud phenomenon. After 15 doses of IV DHE, the patient reported paresthesias of the right hand and was noted to have absent right radial and ulnar pulses to palpation. Portable arterial Doppler study demonstrated abnormal flat line pulse volume recordings (PVRs) in the right second, third, and fourth digits, with markedly dampened PVR in the right thumb and fifth finger along with no ulnar PVR detectable at the wrist. Duplex revealed bilateral severely diminished flow in the right ulnar and radial arteries without acute occlusions. Computed tomography angiogram of right upper extremity visualized arteries through the mid-forearm but not distally. Dihydroergotamine was discontinued, and the patient was started on oral amlodipine and aspirin. Repeat Doppler ultrasound 3 days later revealed normal arm and digital waveforms bilaterally consistent with resolution of vasospasm. This case highlights a potential complication of IV DHE therapy. Risk may be increased in patients with primary Raynaud phenomenon. We suggest cautious use of IV DHE in this population.
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Vaidya, Binit, and Manisha Bhochhibhoya. "Nailfold capillaroscopic findings in patients with Raynaud’s phenomenon: Preliminary report." Journal of Advances in Internal Medicine 4, no. 1 (December 18, 2015): 13–15. http://dx.doi.org/10.3126/jaim.v4i1.14175.
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Back grounds and Aims: Microvascular involvement of nailfold often occurs in systemic rheumatic diseases, especially scleroderma and related conditions. Nailfold capillaroscopy is easily performed non-invasive, simple, repeatable, sensitive, safe and inexpensive method to examine nailfold architecture by microscope.The normal capillaries are thin, symmetrical and have hair-pin appearance. Anomalies include dilated capillary loops, dropouts, tortuosity, avascular area, arborifications, megacapillaries, infracts,thrombsis and haemorrhages. This study aims to see the morphological changes in microcirculation in nailfolds of patients with Raynaud’s, which can be helpful in predicting the presence of connective tissue disorder.Methods: It is an observational study conducted at National Center for Rheumatic Diseases, Kathmandu from 26th March 2014 to 30th March 2015. Nailfolds of 8 fingers of both hands in 105 patients were examined using a hand held microscope.Results: Of 105 patients, 91 patients (86.6%) had abnormal morphological changes while 14 (13.4%) had no significant changes in microvessels. Normal patients had no underlying connective tissue disease(CTD) but those with abnormal findings had proven underlying CTD which included Lupus 6.5%, Scleroderma 17.5%, Rheumatoid Arthritis 14.2%, Mixed CTD7.6%, Undifferentiated CTD1.0%, Undifferentiated inflammatory arthritis 14.2%, Psorasis 1.0%, Rheumatoid Arthritis with scleroderma overlap 4.3%. No primary diagnosis was found in 32.9% of patients.Conclusion: Nailfold capillaroscopy allows direct examination of microvascular structure of a patient. Abnormal findings in a patient with Raynaud’s phenomenon might help to identify the presence of or future evolution into systemic connective tissue disorder.Journal of Advances in Internal Medicine 2015;04(01):13-15
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Kersten, B., K. Daoudi, C. H. M. Van den Ende, F. Van den Hoogen, C. De Korte, and M. Vonk. "AB0582 DIAGNOSING SYSTEMIC SCLEROSIS WITH PHOTOACOUSTIC AND HIGH-FREQUENCY ULTRASOUND IMAGING." Annals of the Rheumatic Diseases 79, Suppl 1 (June 2020): 1588.1–1588. http://dx.doi.org/10.1136/annrheumdis-2020-eular.1388.
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Background:Vasculopathy is already evident in early systemic sclerosis (SSc); Raynaud’s phenomenon and typical nailfoldcapillaroscopic findings are part of the criteria of very early diagnosis of SSc (VEDOSs) (1). As not all early SSc patients have alterations in their nailfoldcapillaries, there is need for other diagnostic tools. Photoacoustics(PA) and high-frequency ultrasound (HFUS) might be able to fulfill this need (2). The former can measure the oxygen saturation of hemoglobin by using short pulsed laser light while the latter can provide high-resolution images that allow measuring skin thickening distal from DIP joint, which could be used to determine skin involvement early.Objectives:We hypothesize that photoacoustics and high-frequency ultrasound can distinguish (early) SSc patients from individuals with primary Raynaud’s phenomenon (PR) by measuring the oxygenation (by PA) of the fingertip and skin thickening (by HFUS).Methods:In our cross-sectional study, we compared measurements of the third finger in (early)SSc patients with individuals with PR and healthy volunteers. Smoking and beta-blockage were exclusion criteria. The level of oxygenation (by PA) and skin thickness (by HFUS) were compared between groups. Nailfoldcapillaroscopy was performed on all subjects and analyzed for the pattern.Results:Thirty-one adult subjects participated in this study: twelve patients with SSc, 5 patients with early SSc, 5 volunteers with PR and 9 healthy controls.We found a significant difference in median (IQR) oxygen saturation between earlySSc patients 75.9% (IQR 75.1%-86.6%) and subjects with PR 94.1% (IQR 93.1%-94.5%) (p=0.0002) using the Wilcoxon rank-sum test (figure 1).Figure 1.Boxplot of saturation (%) per group.Measurements of skin thickening also showed a significant difference in early SSc patients compared to subjects with PR, respectively 0.5mm (IQR 0.4mm-0.5mm) vs. 0.3mm (IQR 0.3mm-0.3mm), P=0.0002 (figure 2).Figure 2.Boxplot of skin thickness (mm) per group.Conclusion:Our results demonstrate that photoacoustic and high-frequency ultrasound can distinguish between (early)SSc and PR in both oxygenation saturation and skin thickening. In a larger prognostic study we want to determine the value of photoacoustic and high frequency ultrasound in diagnosing earlySSc.References:[1]Minier T, Guiducci S, Bellando-Randone S, Bruni C, Lepri G, Czirjak L, et al. Preliminary analysis of the very early diagnosis of systemic sclerosis (VEDOSS) EUSTAR multicentre study: evidence for puffy fingers as a pivotal sign for suspicion of systemic sclerosis. Annals of the rheumatic diseases. 2014;73(12):2087-93.[2]Wang LV, Yao J. A practical guide to photoacoustic tomography in the life sciences. Nature methods. 2016;13(8):627-38Disclosure of Interests:Brigit Kersten: None declared, Khalid Daoudi: None declared, C.H.M. van den Ende: None declared, FHJ van den Hoogen Consultant of: AbbVie, Actelion, Biogen, BMS, Celltrion, Corbus, Eli-Lilly, Mundipharma, Pfizer, Sanofi-Genzyme, Speakers bureau: Amgen, Boehringer-Ingelheim, Novartis, CL de Korte: None declared, Madelon Vonk Grant/research support from: Janssen and Ferrer, Consultant of: Boehringer Ingelheim, Janssen and GSK, Speakers bureau: Boehringer Ingelheim, BMS and Roche
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Obelenis, Vytautas, and Vilija Malinauskienė. "The influence of occupational environment and professional factors on the risk of cardiovascular disease." Medicina 43, no. 2 (November 13, 2006): 96. http://dx.doi.org/10.3390/medicina43020011.
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The article reviews the recent scientific literature and the authors’ studies on this topic. Occupational conditions and psychological factors have been shown to play an important role in the etiopathogenesis of cardiovascular diseases. Their effect is often indirect, through damage to the central nervous, respiratory, and neuroendocrine systems. Hot climate in the workplace and intense infrared radiation cause the water and electrolyte imbalance and chronic hyperthermia and manifests as neurovegetative dystonia. The long-term effects of low temperatures condition ischemic lesions in circulatory system, trophic organ destruction. The influence of ultrahigh-frequency electromagnetic radiation on the cardiovascular system is directly related to the central nervous system and neurohumoral lesions. “Microwave disease” often manifests as polymorphic dystonia. Exposure to occupational vibration causes “white finger” syndrome or Raynaud’s phenomenon together with cerebral vascular lesions. Recent studies have confirmed that noise as a chronic stressor causes the imbalance in the central and vegetative nervous systems and changes in homeostasis. Noise increases catecholamine and cholesterol concentration in blood, has an effect on plasma lipoprotein levels, increases heart rate, arterial blood pressure, and risk of myocardial infarction. Psychophysiological changes caused by long-term stress influence constant pathological changes in the central nervous system, endocrine and cardiovascular systems. The long-term effect of psychogenic stressors is very important in the etiopathogenesis of psychosomatic diseases.
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Çağlayan, Ş., F. Demir, B. Sancar, K. Ulu, T. Coşkuner, and B. Sözeri. "POS1319 RAYNAUD’S PHENOMENON IN A SINGLE CENTER COHORT OF TURKISH CHILDREN." Annals of the Rheumatic Diseases 80, Suppl 1 (May 19, 2021): 942.1–942. http://dx.doi.org/10.1136/annrheumdis-2021-eular.3076.
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Background:Raynaud’s phenomenon (RF) is a vasospastic condition characterized by episodic color changes of blanching, cyanosis, and hyperemia of the extremities of the bodyObjectives:We aim to examine the clinical presentation, capillaroscopic findings and disease associations of Raynaud’s phenomenon (RP) in pediatric patientsMethods:We retrospectively enrolled a single-center cohort of 247 consecutive pediatric patients with RP admitted to Umraniye Training and Research Hospital, Pediatric Rheumatology Clinic, Istanbul, Turkey, since 2016. Medical records were analyzed for clinical presentation, disease associations, and physical examination and laboratory findings.Results:We reported 247 patients (152 female, 95 male) with RP. Their mean age at disease onset was 14.5±2.55 years and median age at diagnosis was 15.4± 2.21 years. In patients with secondary RP syndrome (10.9%), the age of complaint was found to be lower than others (p=0.03). Although most patients have affected upper extremity (hand and fingers), lower extremity (toes) involvement was also detected in 50 (20%) patients. In both the patients with primary and secondary RP, main triggering factor was found as cold exposure. Biphasic (53.6%) color changes were more seen in primary RP, although triphasic (51.8%) color changes were more in patients with secondary RP (p=0.00). The presence of antinuclear antibodies and abnormal nailfold capillaries were more seen in patients with secondary RP (28% vs 74% and 35.3% vs 66.7, respectively p=0.00). Digital ulcer was detected 5.2% of patients (mostly secondary). Out of 247, 31 patients were treated with low-dose aspirin, 47 with calcium blockers, 25 with low-dose aspirin and calcium blockers, 16 with iloprost and/or bosentan, while the remaining 140 did not receive any drug.Conclusion:Similar to adults, RP is more common in girls without an underlying disease. In patients with secondary RP, the symptoms. It has been shown that in patients with secondary RP, symptoms begin at a younger age and the ANA positivity and abnormal nailfold capillaries correlate.References:[1]Jones G, et al. Arthritis Rheum 2003;48:3518–352[2]Garner R et al. BMJ Open. 2015; 5(3): e006389[3]Nigrovic PA et al. Pediatrics 2003, 111 (4) 715-721Disclosure of Interests:None declared
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Opinc, A., O. Brzezińska, and J. Makowska. "SAT0635-HPR THE BURDEN OF SCLERODERMA FROM PATIENTS’ PERSPECTIVE." Annals of the Rheumatic Diseases 79, Suppl 1 (June 2020): 1277.2–1277. http://dx.doi.org/10.1136/annrheumdis-2020-eular.3797.
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Background:Scleroderma can significantly impair daily functioning by leading to multi-organ involvement and profound fatigue. Recent studies suggest that objective measures of the disease severity may not necessarily correspond with the patients’ perception1.Objectives:To evaluate the degree of disability and fatigue in patients with scleroderma, distinguish the most troublesome activities and assess which clinical symptoms had mostly impact on daily functioning.Methods:An online questionnaire was created and distributed to English-speaking patients with scleroderma by means of online support groups. The questionnaire contained inquiries about demographical data, clinical symptoms as well as the Scleroderma Health Assessment Questionnaire (SHAQ, comprised of HAQ-DI and specific VAS scales) and Functional Assessment of Chronic Illness Therapy – Fatigue (FACIT-F). Standard and Alternative Disability Indexes (SDI and ADI) were counted based on answers in HAQ-DI part. Degrees of disability and fatigue, reflected by SDI, ADI and FACIT-F scores, were compared between subgroup of patients with and without particular clinical symptoms. Responses were analysed statistically.Results:Questionnaire was completed by 85 responders (mean age 48,93±14,4; 98,82% were female). Mean SDI was 1.09±0.66 with 41.18% of patients fulfilling the criteria of mild to moderate difficulty (SDI<1), 50.59% classified as moderate to severe disability (1≤SDI<2) and 8.24% being severely or very severely disabled (SDI≥2). Mean value of ADI was 0.93±0.61. 56.47 % of responders needed aid of the caregivers while gripping/opening, 31.76% while reaching and 29.41% while running errands and chores. Most frequently used facilitating devices included devices for gripping/opening (32.94%) and for maintaining hygiene (21.18%). Mean value of overall disease severity was estimated by VAS as 52.06±0.66. The mean values of VAS assessing the impact on daily living were respectively 48.16±29.28 for pain related to the disease, 43.34±34.94 for Raynaud’s, 41.86±34.59 for gastrointestinal problems, 31.60±31.38 for breathing difficulties and 20.85±33.47 for finger ulcers. Mean FACIT-F score was 23.62±11.88 yet 71.76% of patients fulfilled the criteria of severe fatigue. Both SDI and ADI correlated with FACIT-F score (respectively r=-0.5684, p<0.0001 and r=-0.5711, p<0.0001). SDI, ADI and FACIT-F correlated with VAS scores for overall disease severity, pain, gastrointestinal and breathing problems. Patients with symptoms such as arthralgia, muscle weakness, dysphagia, blurred vision, hair loss, unintentional weight loss and hoarseness were statistically more disabled and fatigued as compared to patients without such symptoms. Responders with skin ulcers/non-healing wounds (other than finger ulcers), Raynaud’s or erythema/rash were more disabled than responders without such symptoms, yet no differences were observed in the degree of fatigue.Conclusion:Majority of patients with scleroderma are moderately to severely disabled yet cope with the impairments with the widespread use of facilitating devices or aid of caregivers. Profound fatigue, observed in the majority of patients, may contribute to the high overall perception of disease severity.References:[1]Jaeger VK, Distler O, Mauer B et al. Functional disability and its predictors in systemic sclerosis: a study from the DeSScipher project within the EUSTAR group. Rheumatology (Oxford). 2018 Mar 1;57(3):441-450. doi: 10.1093/rheumatology/kex182Disclosure of Interests:None declared
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Krabbe, Simon, Merete Engelhart, Sören Thybo, and Søren Jacobsen. "Mycobacterium intracellulare Infection Mimicking Progression of Scleroderma." Case Reports in Rheumatology 2017 (2017): 1–3. http://dx.doi.org/10.1155/2017/4029271.
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This case report describes a patient with scleroderma who developed Mycobacterium intracellulare infection, which for more than a year mimicked worsening of her connective tissue disorder. The patient was diagnosed with scleroderma based on puffy fingers that developed into sclerodactyly, abnormal nail fold capillaries, interstitial lung disease, Raynaud’s phenomenon, esophageal dysmotility, and positivity for rheumatoid factor and anti-SSA antibodies. She developed massive inflammatory changes of the cutis, the subcutis, and the muscle fasciae of the right leg, that after several failed attempts of immunosuppressive treatments were found to be caused by Mycobacterium intracellulare. While she was receiving high-dose prednisolone, as worsening of her connective tissue disease was suspected to be the cause of the inflammatory changes, she had Listeria monocytogenes meningitis and was hospitalized for several weeks, but she recovered from this without sequelae. After Mycobacterium intracellulare infection was diagnosed, she was treated with clarithromycin and rifampicin. Her skin manifestations, arthralgias, and fatigue improved considerably, and the wounds of the right leg healed, unfortunately with significant scarring. Immunodeficiency testing was unremarkable. In summary, an infection with Mycobacterium intracellulare was mistaken for an unusually severe progression of scleroderma.
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Paradowska-Gorycka, Agnieszka, Anna Wajda, Barbara Stypinska, Ewa Walczuk, Marcela Walczyk, Anna Felis-Giemza, Aleksandra Poluch-Lewandowska, and Marzena Olesińska. "Interferons (IFN-A/-B/-G) Genetic Variants in Patients with Mixed Connective Tissue Disease (MCTD)." Journal of Clinical Medicine 8, no. 12 (November 21, 2019): 2046. http://dx.doi.org/10.3390/jcm8122046.
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Mixed connective tissue disease (MCTD) is a rare complex autoimmune disease in which autoantigens are recognized by endosomal TLRs. Their activation induces a higher secretion of the type I interferons, IFN-γ and the up-regulation of the INF-inducible genes. The present study aimed to investigate whether SNPs that are located in the IFN-A, IFN-B, and IFN-G genes are associated with MCTD. 145 MCTD patients and 281 healthy subjects were examined for IFN-A, IFN-B, and IFN-G genetic variants by TaqMan SNP genotyping assay. ELISA determined IFN-α/-β/-γ serum levels. Among the seven tested SNPs, four polymorphisms: IFN-A rs10757212, IFN-A rs3758236, IFN-G rs2069705, IFN-G rs2069718, as well as INF-G rs1861493A/rs2069705A/rs2069718G haplotype were significantly associated with a predisposition for MCTD. Raynaud’s phenomenon, erosive arthritis, swollen hands and fingers, and sclerodactyly were significantly more frequently observed in MCTD patients with IFN-G rs2069718 G allele than in patients with IFN-G rs2069718 A allele. We also found that anti-U1-A autoantibodies most frequently occurred in MCTD patients with rs2069718 GA genotype, while the IFN-G rs2069705 AG and rs2069718 GA genotypes might be a marker of anti-Ro60 presence in MCTD patients. Our results indicate that IFN-G genetic variants may be potential genetic biomarkers for MCTD susceptibility and severity.
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Siqueira, V., M. Helbingen, A. P. Luppino-Assad, H. Carriço Da Silva, D. Andrade, A. C. Medeiros-Ribeiro, and P. D. Sampaio-Barros. "SAT0341 PREDICTORS TO PROGRESSION TO SYSTEMIC SCLEROSIS IN A GROUP OF SECONDARY RAYNAUD PHENOMENON OBSERVED IN A LARGE SINGLE BRAZILIAN COHORT." Annals of the Rheumatic Diseases 79, Suppl 1 (June 2020): 1116.1–1116. http://dx.doi.org/10.1136/annrheumdis-2020-eular.4220.
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Background:The 2013 ACR/EULAR classification criteria for systemic sclerosis (SSc) allowed the inclusion of a subset of patients without skin involvement, emphasizing the need of early diagnosis of SSc.Objectives:In this setting, the objective of this study was to classify, according to ACR/EULAR SSc criteria, patients with Raynaud phenomenon (RP) and at least one SSc manifestation, except for skin involvement, and analyze predictors for development of SSc.Methods:This is a cross-sectional single-center analysis of consecutive patients presenting RP and a characteristic SSc manifestation (SSc autoantibody, SSc visceral involvement or SD pattern at nailfold capillaroscopy - NFC), without skin involvement, who attended a scleroderma outpatient clinic between 2010 and 2019. Patients were classified as SSc (according to ACR/EULAR SSc criteria) or undifferetiated connective tissue disease (UCTD) in their last medical visit and compared. Additionally, a longitudinal retrospective analysis of both groups was performed to find predictors for development of SSc. Data were obtained from an electronic register database. Statistical significance was set up as p<0.05.Results:Among 217 patients, 153 (70.5%) were classified as SSc, at the last medical visit, including 65 (30%) after the first investigation. During a comparable median follow-up [4.9±3.8 vs. 4.6±3.0 years;p=0.90], patients with SSc presented more frequently puffy fingers (PF:73% vs. 6.3%;p<0.01), pitting scars (87.4% vs. 4.7%;p<0.01), digital ulcers (25.2% vs. 1.6%;p<0.01), telangiectasias (52.3% vs. 7.8%;p<0.01), interstitial lung disease (ILD:66.7% vs. 34.4%;p<0.01), pulmonary hypertension (PH:18.3% vs. 7.9%;p=0.049) and esophageal dismotility (ED: 58.9% vs. 43.8%:p=0.02) than secondary RP patients without SSc. Moreover, SSc patients had more SD pattern at NFC (83.4%vs. 57.8%;p<0.01), anti-Scl70 antibody (16.6%vs. 3.1%;p<0.01), and nucleolar ANA pattern (22.5%vs. 10.9%;p=0.048). Furthermore, SSc patients were more frequently treated with immunosuppressive therapy (80.1%vs. 37.5%;p<0.01) and presented an increased death rate (9.9%vs. 1.6%;p<0.01 and Figure 1). For the analysis of the predictors to SSc, patients who had already fulfilled SSc criteria at baseline were excluded and 152 patients were analysed: 88 progressed to SSc and 64 remained as UCTD. After multivariate analyses, puffy fingers (OR=4.48; 95%CI 1.17-17.17;p=0.029) and a higher SSc score, both at baseline, (OR: 1.97; 95%CI 1.33-2.92;p<0.001) were predictors to SSc, while esophageal symptoms (OR=0.18; 95%CI 0.05-0.67; p=0.01) and absence of clinical symptoms (OR=0.08; 95%CI 0.01-0.40; p=0.002) were negative predictive factors. When analyzing the combination of SSc features, RP + PF + positive ANA (OR=15.5; 95%CI 4.5-53.1;p<0.001), RP + PF + positive ANA + SD-NFC (OR=12.3; 95%CI 2.8-54.2;p<0.001), RP + PF + SD-NFC (OR=12; 95%CI 2.79-54.1;p<0.01), and RP + lung disease (ILD/HAP) + SD-NFC (OR=3.0; 95%CI 1.1-10.5;p<0.04) were associated with classification as SSc, while combinations without non-RP clinical symptoms, as RP + SD-NFC (OR=0.027; 95%CI 0.003-0.210;p<0.001) and RP + anticentromere + SD-NFC (OR=0.050; 95%CI 0.007-0.440;p<0.001) were not associated with SSc.Conclusion:Among patients with RP and SSc features, but without skin involvement, those with SSc had a more severe disease with worse prognosis than UCTD with RP. Puffy fingers and a higher baseline SSc score, especially in combinations including PF or lung disease were predictors of SSc, reinforcing the strength of the ACR/EULAR classification criteria and the presence of specific non-RP symptoms.Disclosure of Interests:Valdirene Siqueira: None declared, Mariely Helbingen: None declared, Ana Paula Luppino-Assad: None declared, Henrique Carriço da Silva: None declared, Danieli Andrade: None declared, Ana Cristina Medeiros-Ribeiro: None declared, Percival D. Sampaio-Barros Consultant of: Abbvie, Boehringer Ingelheim, Lilly, Novartis, Speakers bureau: Abbvie, Janssen, Lilly, Novartis
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Azuma, N., T. Furukawa, Y. Shima, and K. Matsui. "FRI0227 A USABILITY SURVEY OF WRIST MOUNTED DISPOSABLE HEAT PAD ON RAYNAUD’S PHENOMENON IN PATIENTS WITH CONNECTIVE TISSUE DISEASES." Annals of the Rheumatic Diseases 79, Suppl 1 (June 2020): 696.1–697. http://dx.doi.org/10.1136/annrheumdis-2020-eular.443.
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Background:For patients with connective tissue diseases (CTD), vasodilators are used to treat Raynaud’s phenomenon (RP), they are difficult to control only by medication. Although physicians recommend the use of a portable handwarmer or gloves to patients with CTD presenting with RP, sustained heat-retention effects cannot be obtained from them because the patients’ daily life-related activities prevent their continued use. Since the wrist mounted disposable heat pad maintains the degrees of freedom of the hands and fingers and can remain usable during the daily activities, we considered this heat pad as a useful and highly practical heating method for CTD patients presenting with RP.Objectives:To investigate the usability and changes in symptoms resulting from the use of the wrist mounted disposable heat pad in CTD patients presenting with RP.Methods:Subjects were 23 outpatients with CTD presenting with RP (23 females; mean age 62.6 years; mean duration following the onset of RP 10.3 years; 12 systemic sclerosis, 5 mixed connective tissue disease, 5 Sjögren’s syndrome, and 1 systemic lupus erythematosus) who had used the wrist mounted disposable heat pad (put the pad in a specifically designed holder and wrap it around wrist joint (max. temperature 42 degrees Celsius, heat-retention time 6 hours)). We investigated through interviews with them the use situations, usability, and changes in RP. During their using the heat pad, medication and daily life-related precautions against RP continued to be implemented as before.Results:Many patients had no knowledge of the heat pad (n=17, 73.9%). The most common wearing time of the heat pad was 5–6 hours (n=8, 34.8%). As for scenes of wearing the heat pad, patients who wore the pad when being out of the home accounted for the highest proportion (n=16, 69.6%), and as follows: at home (n=6, 26.1%), during kitchen work (n=3, 13.0%), and during housework (n=2, 8.7%). 17 patients (73.9%) replied that usability was “good”, and 18 (78.3%) replied that usability was “better” compared with conventional measures. Moreover, many patients (n=16, 69.6%) replied that RP and associated symptoms had become reduced or alleviated. No patients replied that RP and associated symptoms had become exacerbated or severer. In terms of advantages of using the heat pad, patients who replied that the site on which the pad was mounted was felt to be warm accounted for the highest proportion (n=8, 34.8%), and those who replied that sites other than where the pad was mounted (such as fingertips, hands, and arms) were also warmed accounted for virtually the same proportion (n=7, 30.4%). Over 60% of the patients (n=14, 60.9%) replied that symptoms associated with RP (skin color, cold sensation, and pain) had become reduced or disappeared. In terms of disadvantages of using the heat pad, patients who replied that it was bothersome to use the pad accounted for the highest proportion while other patients made replies referring to cost and bad appearance. No significant accident occurred and as many as 17 patients (73.9%) replied that they would like to continue to use the heat pad in the future.Conclusion:There have been few reports evaluating the usefulness of a heat pad for RP. The wrist mounted disposable heat pad was thought to be a heating method having the potential to achieve high levels of usability and practicality on CTD patients presenting with RP. Given that the heat pad alleviated RP or caused sites other than where the pad was mounted to be felt warm even though it did not directly heat the hands and fingers, the pad seemed to have usefulness attributed to the heating of the wrist. Although the heat pad seems to be an excellent method for addressing RP in patients’ daily lives, we hope that this heat pad will be evaluated on a larger number of patients with the addition of objective indices.References:[1]Koscheyev VS, et al. Aviat Space Environ Med. 72: 713-719, 2001.Disclosure of Interests:Naoto Azuma: None declared, Tetsuya Furukawa: None declared, Yoshihito Shima Grant/research support from: Endowed chair funded by/accepted a researcher from Kirikai Chemical and Kobayashi Pharmaceutical., Kiyoshi Matsui Grant/research support from: Asahi Kasei Pharma, Astellas Pharma (research grants), Speakers bureau: Bristol-Myers Squibb (lecture fees)
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Bello, S., A. Rinaldi, S. Trabucco, L. Serafino, C. Bonali, and G. Lapadula. "Erasmus syndrome in a marble worker." Reumatismo 67, no. 3 (February 9, 2016): 116. http://dx.doi.org/10.4081/reumatismo.2015.826.
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Erasmus syndrome is defined as the association of silica exposure and subsequent development of systemic sclerosis. The limited number of cases reported in the literature mainly involves miners and only sporadically other professionals. We describe a case of Erasmus syndrome in a marble worker. A 68 year old man came to our observation complaining pelvic and scapular girdle pain, evening fever, intense weakness and emaciation for about 1 month. He also reported to have had Raynaud’s phenomenon in his hands for the last 13 years. Also, his occupational history revealed a chronic exposure to silica dust. The patient presented pain in his shoulders and hips, moderate skin thickening and sclerosis in his hands and fingers extending proximally to his wrists. The diagnosis of systemic sclerosis was determined according to his clinical and medical history, the positivity of anti-Scl 70 antibodies, the nailfold capillaroscopy suggestive of an active scleroderma pattern and the detection of a mild restrictive pulmonary syndrome. The evaluation of the organbased complications excluded a gastroenterological and cardiovascular involvement, while the chest computed tomography (CT) detected multiple small nodules with a mantle distribution and enlarged lymph nodes with no signs of interstitial lung disease and fibrosis. Additional tests (positron emission tomography-CT, flexible bronchoscopy and broncho-alveolar lavage) excluded infectious diseases and cancer. However, given the pulmonary involvement, we performed a histological examination of the parenchyma and lymph nodes, which revealed a picture of pneumoconiosis. In the end, the occupational history and the findings from the diagnostic procedures led to the diagnosis of pulmonary silicosis. The precise definition of the pulmonary involvement was essential to the therapeutic approach to this patient.
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Desinova, O., M. Starovoytova, and L. P. Ananyeva. "AB0569 SPECIFIC FEATURES OF VASCULAR DISORDERS IN SYSTEMIC SCLEROSIS-OVERLAP SYNDROMES." Annals of the Rheumatic Diseases 79, Suppl 1 (June 2020): 1581. http://dx.doi.org/10.1136/annrheumdis-2020-eular.4401.
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Background:The Systemic Sclerosis-overlap syndromes are a typical representative of the well-known group of overlap syndromes in rheumatology, causing great difficulties in diagnostic and therapeutic aspects.Objectives:To identify specific features of vascular disorders in patients with Systemic Sclerosis-overlap syndromes.Methods:100 patients (pts) were followed, including 68 pts with Systemic Sclerosis-polymyositis/dermatomyositis (SSc-PM/DM) (f:m 52: 16), aged 17 to 74 years (mean 43±13.9) and mean disease duration 5 [2-7] years, and 32 pts with Systemic sclerosis-Rheumatoid arthritis (SSc-RA) (f:m 31:1), aged 17 to 74 years (mean age 48±14.8) and average disease duration 11.5 [5-18,5] years. The comparison group consisted of 100 pts with Systemic sclerosis (SSc) (f:m 90:10), aged 51 to 75 years (mean 51±12.9) and mean disease duration 10.6 [5-13,5] years.Results:In 1/3 of pts with SSc-PM/DM, the Raynaud’s phenomenon (RP) preceded the emergence of skin lesions and was the first symptom of the disease (33%), developing in the first year in the majority (68%) of cases, being a unique sign of the disease in only 6 (9 %) pts, while in all other cases it was associated with articular involvement, skin lesions and muscle disease. In the first year of the disease 7 pts (10%) already had signs of digital ischemia (scars, ulcers), and by the end of 3 years such signs were present in 1/3 of SSc-PM/DM pts (32%). 29 pts (43%) developed recurrent digital trophic disorders, such as digital scars in 12 (18%), digital ulcers in 16 (23.5%) and digital necrosis of the distal phalanx in 1 pts (1.5%).In 25 (78%) SSc-RA pts there was a gradual, monosymptomatic – with isolated RP - onset of the disease, similar to chronic cutaneous cSSc. In 29 SSc-RA (91%) pts, RP developed in the first year, and in 23 (72%) it was the first sign of the disease, and in 12 (37.5%) it preceded the development of skin and joint symptoms. Vascular disorders progressed with increasing duration and activity of the process. 7 patients (22%) developed recurrent digital trophic disorders - predominantly finger scars, while 3 (9%) pts had digital ulcers, developing in 11.66±7.62 years on average from the onset of the disease. Correlation analysis revealed a link between patients’ age of and presence of telangiectasias (p<0.02). Digital trophic disorders were significantly more frequent in patients with longer disease duration (p<0.05).Comparing of SSc-overlap syndromes and SSc pts showed, that although RP was present in all of them, nevertheless digital vascular-trophic disorders such as scars, ulcers (p<0.02) and necrosis (p<0.001) were less likely to develop in pts with SSc-overlap syndromes, similarly, telangiectasia and osteolysis were also significantly less frequent (p=0.04) distinguishing SSc-overlap syndromes from limited SSc (Table). Although, SSc-overlap syndromes pts had more severely affected muscles and joints as compared to SSc.Table 1.Comparative characteristics of vascular disorders in SSc and SSc patientsAnalyzed featuresSScn=100 (%)SSc- overlap syndromesn=100 (%)SSc-PM/DMn= 68 (%)SSc-RAn=32 (%)Raynaud phenomenon100 (100)100 (100)68 (100)32 (100)Digital trophic disorders: scars19 (19)19 (19)12 (18)7 (22) ulcers34 (34)**19 (19)**16 (23,5)3 (9)* necrosis16 (16)***1 (1)***1 (1,5)***0 (0)***Telangiectasia60 (60)*46 (46)*34 (50)12 (37,5)*Osteolysis37 (37)*24 (24)*16 (23,5)8 (25)* р<0,05, ** р<0,03, *** р<0,001Conclusion:Digital trophic disorders (ulcers, necrosis), as well as telangiectasia and osteolysis are significantly less likely to develop in SSc-overlap syndromes than in SSc, which should be taken into account when panning therapeutic strategies in this category of patients.Disclosure of Interests:None declared
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Blaja, Elisabeth, Suzana Jordan, Carmen-Marina Mihai, Rucsandra Dobrota, Mike Oliver Becker, Britta Maurer, Marco Matucci-Cerinic, and Oliver Distler. "The Challenge of Very Early Systemic Sclerosis: A Combination of Mild and Early Disease?" Journal of Rheumatology 48, no. 1 (March 15, 2020): 82–86. http://dx.doi.org/10.3899/jrheum.190976.
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Objective.To address the hypothesis that very early patients with systemic sclerosis (SSc) are a heterogeneous group with mild or early disease, we analyzed the extent of heterogeneity in clinical, epidemiological, and immunological characteristics of these patients.Methods.We performed an analysis of very early SSc patients from the Zurich cohort, who fulfilled neither the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism nor the 1980 ACR classification criteria, but had a clinical expert diagnosis of SSc with Raynaud phenomenon (RP) and additional features of SSc (puffy fingers, SSc-specific antibodies, SSc pattern on nailfold capillaroscopy, or any organ involvement characteristic for SSc). Disease duration was defined from first RP symptom.Results.One hundred and two patients fulfilled the inclusion criteria and were analyzed. Their clinical presentation was heterogeneous with the large majority presenting with RP, antinuclear antibodies, and nailfold capillaroscopy changes, but with varying presentations of other features such as SSc-specific antibodies and early signs of organ involvement. While 54.1% (52/96) of patients had a disease duration of < 5 years, as many as 29.1% (28/96) of patients had a disease duration of > 10 years, indicating long-standing mild disease. Patients with very early, potentially progressive disease did not differ from patients with long-standing mild disease in terms of their clinical features at first presentation.Conclusion.This study showed that patients with very early SSc are a mixture with mild or early disease. This needs to be considered in clinical practice for risk stratification and for the study design of patients considered as early SSc.
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Dhir, V., S. Jha, A. Sharma, S. Jain, S. K. Sharma, and G. Naidu. "AB0570 MIXED CONNECTIVE TISSUE DISEASE: NOT THAT UNCOMMON, A SINGLE-CENTER EXPERIENCE FROM INDIA." Annals of the Rheumatic Diseases 79, Suppl 1 (June 2020): 1582.2–1582. http://dx.doi.org/10.1136/annrheumdis-2020-eular.5530.
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Background:Mixed connective tissue disease (MCTD) is considered to be uncommon; specifically there is sparse data on MCTD from developing countries like India.Objectives:This study examines the clinical and serological features of these patients in a single center in North-India.Methods:This was a retrospective single-center study of patients diagnosed as MCTD in last 20 years. The patients included fulfilled at least one of the diagnostic criteria namely Alarcón-Segovia, Kasukawa, and Kahns. Demographic details, clinical signs and symptoms, laboratory parameters, treatment and outcome were extracted from medical records and clinic files in a pre-designed proforma.Results:This study included 41 MCTD patients. There was a marked female preponderance (F: M=40:1), and mean age of disease onset and diagnosis was 33.8 ± 10.7 and 39.3 ± 10.2 years. 39 (92%) of the patients fulfilled both Kahn and Kasukawa criteria, while 31 (76%) fulfilled Alarcon-Segovia criteria. Initially patients had been (mis)diagnosed as rheumatoid arthritis, systemic lupus erythematosus (or UCTD) (in five patients each), overlap syndromes or myositis (in 4 patients). ANA was commonly high-titer and specked, U1RNP was positive in all. (Table 1) Other autoantibodies on immunoblot included SSA and Ro52 in half the patients. Raynaud’s was seen in three-fourth at presentation and all the patients over time. Digital gangrene and puffy fingers were seen in 8 (20%) and 18 (46%) patients. Other clinical features included arthritis in 33 (81%), sclerodactyly in 23 (56%) and proximal weakness in 20 patients (49%). Interstitial lung disease and pulmonary arterial hypertension were seen in 20 (57%) and 15 (44%) patients. All patients (except one) received prednisolone, and it was currently used in almost 90%. Intravenous cyclophosphamide was used in one-third, commonly for ILD.Table 1.Laboratory features of patients with MCTDLabs n (%)Leucopenia9 (22)Thrombocytopenia10 (24)Raised globulinsb22 (69)Mean globulins, mean (SD)a5±3.4Elevated CPKb10 (31)CPK Levels U/L, median (IQR)256 (57.5-1036)ANA Speckled Pattern N (%)31d(82)U1RNP N (%)b32 (100%)U1RNP Blot Intensity1+8 (25%)2+1 (3.2%)3+7 (21.8%)4+16 (50%)U1RNP EIA, mean(SD), n=19141.3 ± 82.4Raised RF Titersh10 (35.7%)Low C3 mg/dlf8 (40%)Low C4 mg/dlf3 (15%)FVC, mean (SD) n=2082.4 ± 18.9 ILD on HRCTg20 (57) Dilated PA on CTf10 (50)PAHeon ECHO15 (44.1%)RA/RV Dilated5 (18)SD-standard deviation, IQR- Interquartile range, CPK- Creatinine phosphokinase,, ILD- Interstitial lung disease, PAH- Pulmonary arterial hypertension, ANA- Anti nuclear antibody, IIF- Indirect Immunofluorescence, PA- pulmonary artery, RA/RV- Right atrium/Right Ventricle,a34b32d38e34f20g34h28Conclusion:MCTD was not uncommon in the single-center in North India. Kahn and Kasukawa criteria were found to be the most sensitive for its diagnosis. Digital gangrene was relatively common and sometimes the presenting feature; whereas puffy fingers was present in only half the patients.Disclosure of Interests:None declared
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Uslu, S., S. Gulle, A. Koken Avsar, A. Karakas, S. B. Kocaer, T. Yüce İnel, Y. Erez, et al. "SAT0344 LIMITED JOINT MOBILITY OF HAND IN SYSTEMIC SCLEROSIS PATIENTS BY USING “PRAYER” AND “TABLE TOP” SIGNS." Annals of the Rheumatic Diseases 79, Suppl 1 (June 2020): 1117.1–1118. http://dx.doi.org/10.1136/annrheumdis-2020-eular.4494.
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Background:Limited joint mobility (LJM) is a musculoskeletal disorder caused by flexion contractures of hand is a common complication in systemic sclerosis (SSc) patients. The distal parts of the upper limb (hands and fingers) is the most involved site in SSc.Objectives:In this study, we aimed to evaluate LJM in SSc patients and to determine the relationship between the clinical features of the disease.Methods:A total of 113 patients (>18 years old) diagnosed with diffuse cutaneous systemic sclerosis (DcSSc) and limited cutaneous systemic sclerosis (LcSSc) and 104 healthy controls were included in this study. LJM was evaluated with “prayer sign” and “table top sign” tests. LJM staging was done by Rosenbloom classification method(1, 2). LJM (+) and LJM (-) patients were compared in terms of demographic findings (gender, age and duration of disease), laboratory results (ESR, CRP, ANA, anti-topoisomerase I and anti-centromere) and modified Rodnan Skin Score (mRSS) results.Results:In our study, a total of 113 patients diagnosed with SSc and 104 healthy controls with similar age and gender distribution were included. While LJM (+) was detected in 75 (66.4%) (LcSSc = 38, DcSSc = 37) of the patients diagnosed with SSc, LJM (mild) (+) was detected in 3 (2.8%) of the control group. One of these people had right 2nd DIF joint contracture due to osteoarthritis, and 1 patient was found to have simple contractures due to minor hand injury previously (Table 1). A statistically significant difference was observed in between LcSSc and DcSSc patients according to the presence of LJM (p<0.001) (Table 2). There was a moderate positivity relationship between LJM and mRSS (LcSSc: r=0.449 ve p<0.001, DcSSc: r= 0.565 ve p<0.001) (Figure 1).Table 1.Comparison of demographic findings between SSc and Control groupSSc Group (n=113)Control Group (n=104)p valueAge, year57.02 ± 11.5858.47 ± 11.260.061Gender (F / M)98 (86.7) / 15 (13.3)65 (62.5) / 39 (37.5)0.064CRP (mg/L)5.45 ± 5.392.14 ± 1.12<0.001ESR (mm/hr)25.19 ± 18.914.46 ± 10.090.024Smoking, n (%)Smoker89 (78.8)70 (67.3)0.464Non-Smoker24 (21.2)34 (32.7)LJM (Absent / Present)Present75 (66.4)3 (2.8)<0.001Absent38 (33.6)101 (97.2)Rosenbloom classificationLcSSc (n=71) (%)DcSSc (n=42) (%)Total (n=113) (%)Normal46.511.933.6Mild22.514.319.5Moderate23.933.327.4Severe7.140.519.5Table 2.Comparison of demographic and clinical findings LJM(-) and LJM(+) in SScLJM (-) (n=38)LJM (+) (n=75)p valueAge, year54.16 ± 11.8258.47 ± 11.260.061SSc Typen (%)n (%)LcSSc, n (%)DcSSc, n (%)33 (56.8)38 (50.7)<0.0015 (13.2)37 (49.3)Gender,F/M (%)37 (97.3) / 1 (2.7)61 (81.3) / 14 (18.7)0.018Raynaud’s (symptom duration), month148 (44-456)150 (35-588)0.990Non-raynaud (symptom duration), month108 (28-458)138 (38-447)0.132mRSS, median2 (0-14)8 (0-36)<0.001CRP (mg/L)4.21 ± 4.486.08 ± 5.710.069ESR (mm/hr)19.74 ± 1027.95 ± 21.60.270Renal crisis, n (%)1 (2.6)4 (5.3)0.662PAH, n (%)8 (21.1)14 (18.7)0.762ANA positivity, n (%)36 (94.7)70 (93.3)1Anti-centromere positivity, n (%)18 (47.4)19 (25.3)0.01Anti-topoisomerase-1, n (%)8 (21)34(45.3)0.01Smoking, n (%)n (%)n (%)Non-smoker30(78.9)59 (78.7)0.970Smoker8 (21.1)16 (21.3)Figure 1.Conclusion:In our study, it was found that LJM staging positively correlated with mRSS and DcSSc patients had more severe LJM findings than LcSSc. We conclude that “prayer sign” and “table top sign” tests used in hand evaluation in SSc patients have similar clinical results with mRSS and can be easily performed in daily practice in about 3 minutes.References:[1]Rosenbloom AL. Limitation of finger joint mobility in diabetes mellitus. The Journal of diabetic complications 1989; 3: 77-87.[2]Nashel J, Steen V. Scleroderma mimics. Current rheumatology reports 2012; 14: 39-46.Disclosure of Interests:None declared
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Fujii, Yuichi, Shinji Kishimoto, and Yukihito Higashi. "Finger blood flow after the cold challenge with primary Raynaud’s syndrome: a case report." European Heart Journal - Case Reports, December 1, 2020. http://dx.doi.org/10.1093/ehjcr/ytaa394.
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Abstract Background Raynaud’s syndrome is a commonly encountered disorder. The relationship between the grade of Raynaud’s phenomenon and severity of vasoconstriction is unclear. Recently, various methods including colour Doppler ultrasonography have been used for assessment of vascularity of the extremities including fingers. Case summary A 53-year-old man had a 6-year history of Raynaud’s phenomenon with typical tri-coloured changes proceeding from white, blue to red and slight pain and slight paresthaesia in the fingers of both hands when his fingers were exposed to cold. He was diagnosed with primary Raynaud’s syndrome. After treatment with the calcium channel blocker amlodipine (5 mg once daily), a cold challenge did not induce Raynaud’s phenomenon on the fingers in the present patient. After the cold challenge, colour Doppler ultrasonography showed that vascularity was markedly decreased or was absent, whereas there was little difference in skin colour of the fingers. Discussion In Raynaud's phenomenon, vasospasm may occur even if the symptoms are well-controlled with a calcium channel blocker. It is unlikely that clinical symptoms in patients with Raynaud’s syndrome always reflect the severity of vasoconstriction in their fingers.
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Daoudi, Khalid, Brigit E. Kersten, Cornelia H. M. van den Ende, Frank H. J. van den Hoogen, Madelon C. Vonk, and Chris L. de Korte. "Photoacoustic and high-frequency ultrasound imaging of systemic sclerosis patients." Arthritis Research & Therapy 23, no. 1 (January 12, 2021). http://dx.doi.org/10.1186/s13075-020-02400-y.
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Abstract Introduction Systemic sclerosis starts with an early phase characterized by Raynaud’s phenomenon, puffy fingers/hands, autoantibodies, and a scleroderma nailfold microscopic pattern. Alterations in the nailfold microscopic pattern are not evident in all early SSc patients. Photoacoustics (PA) and high-frequency ultrasound (HFUS) could fulfill this need. The former can measure oxygen saturation while the latter can measure skin thickening. We hypothesize that photoacoustics and high-frequency ultrasound can distinguish (early) SSc patients from individuals with primary Raynaud’s phenomenon (PRP) by measuring oxygenation of the fingertip and skin thickening. Methods We compared measurements of oxygenation and skin thickness of the third finger between (early) SSc patients and PRP individuals and healthy controls. The spearman rank correlation was used to analyze an association between capillary density and oxygen saturation of the fingers. Results Thirty-one adult subjects participated in this study: twelve patients with SSc, 5 patients with early SSc, 5 volunteers with PR, and 9 healthy controls. We found a significant difference in oxygen saturation between (early) SSc patients (80.8% ± 8.1 and 77.9% ± 10.5) and individuals with PRP (93.9% ± 1.1). Measurements of skin thickening showed a significant difference in (early) SSc patients compared to individuals with PRP (0.48 ± 0.06 mm and 0.51 ± 0.16 mm vs. 0.27 ± 0.01 mm). There was no significant difference between healthy and PRP individuals in oxygenation or skin thickening. Conclusion Photoacoustic and high-frequency ultrasound could help to distinguish between (early) SSc, PRP, and healthy individuals in both oxygenation and skin thickening.
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Urban, Nikolaus, Benedikt Weber, Julia Deinsberger, Michael Gschwandtner, Wolfgang Bauer, and Alessandra Handisurya. "Raynaud’s Phenomenon after COVID-19 Vaccination: Causative Association, Temporal Connection, or Mere Bystander?" Case Reports in Dermatology, September 15, 2021, 450–56. http://dx.doi.org/10.1159/000519147.
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Herein, we report a case of a new-onset Raynaud’s phenomenon (RP), which occurred in an otherwise healthy 31-year-old Caucasian woman, who lacked any known risk factors and associations with possible causes for secondary RP. However, 2 weeks prior to the development of RP, the patient had received her first injection of the COVID-19 vaccine containing ChAdOx1-SARS-COV-2. The patient presented with well-demarcated, white-pale, cold areas involving the middle fingers of both hands and the ring finger of the right hand, which were triggered by exposure to cold environment and accompanied by a sensation of numbness. Infrared thermography revealed notable temperature differences of up to 10.9°C between affected and nonaffected fingers. Coagulation and immunological parameters, including cryoglobulins and pathological autoantibodies, were within the normal range and antibodies to the heparin/platelet factor 4 complex not detectable. It remains unclear if the development of RP in our patient is causally related to antecedent COVID-19 vaccination; however, the temporal connection to the vaccination, the complete absence of RP in her past medical history, and the lack of any risk factors and triggers raise the suspicion of a yet unknown association with the vaccine. Whether a clear association between the development of RP and COVID-19 vaccination exists or whether RP represents a bystander effect needs to be awaited in case observational reports on RP accumulate. Given the steadily rising numbers of people receiving COVID-19 vaccinations, physicians may remain alert to still unrecognized side effects.
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Butt, Zaran A., Wan Lin NG, Donough Howard, and Kamal Osman. "P033 Scleroderma-like fibrotic disorder associated with a gastro-intestinal stromal tumour which responded only to imatinib." Rheumatology 60, Supplement_1 (April 1, 2021). http://dx.doi.org/10.1093/rheumatology/keab247.030.
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Abstract Background/Aims Gastro-intestinal stromal tumour (GIST) is recognised as the most prevalent mesenchymal tumour of the gastro-intestinal (GI) tract. The vast majority of GIST display activating mutations in receptor tyrosine kinases, such as platelet-derived growth factor receptor alpha (PDGFRA) and c-kit. This characteristic has lead to the development and approval of targeted therapies such as Imatinib for metastatic GIST. Scleroderma is an idiopathic fibrotic syndrome which involves excess collagen deposition in multiple tissues and organs. There is emerging evidence that dysfunctional fibroblasts are central to the underlying pathogenesis of this disease. An increasing awareness of the complex interplay of these fibroblasts with other cells and inflammatory mediators, such as platelet-derived growth factor (PDGF), is being recognised. This novel case serves to further develop our understanding of these pathological interactions, and to highlight a prospective role for Imatinib in disrupting them. Methods We present a case report below. Results A 54-year-old Caucasian male was referred to our rheumatology clinic due to worsening Raynaud's syndrome, arthralgia, and dry cough. He subsequently developed weight loss, pyrosis and skin thickening. This clinical picture was suggestive of a new diagnosis of systemic sclerosis. However, fibrotic skin changes did not extend to the distal finger, and anti-nuclear antibody (ANA) was negative. Symptoms were also refractory to various vasodilator and immunosuppressive therapies, including nifedipine, repeated iloprost infusions, azathioprine, mycophenolic acid, prednisolone, and sildenafil. Computed tomography (CT) imaging revealed interstitial pulmonary fibrosis and a mass within the gastric fundus. Histopathologic features and immunological staining of this mass were consistent with a GIST. Resection of the tumour did not improve fibrotic symptoms. Surveillance imaging one-year later was highly concerning for metastatic recurrence, which was later confirmed with tissue biopsy. He was subsequently initiated on Imatinib therapy, which led to a rapid improvement in fibrotic changes within weeks. Conclusion While there have been previous descriptions of para-neoplastic fibrotic syndromes, this is the first reported case of a scleroderma-mimicking disorder associated with GIST. It hints at an important potential overlap in the pathogenesis of these disease processes, and a potential role for tyrosine-kinase inhibitors in the treatment of scleroderma-like fibrotic disorders. Disclosure Z.A. Butt: None. W. Ng: None. D. Howard: None. K. Osman: None.