Log in

Relevant bibliographies by topics / Progressive systemic sclerosi / Journal articles

To see the other types of publications on this topic, follow the link: Progressive systemic sclerosi.

Journal articles on the topic 'Progressive systemic sclerosi'

Author: Grafiati

Published: 11 March 2023

Create a spot-on reference in APA, MLA, Chicago, Harvard, and other styles

Select a source type:

Consult the top 50 journal articles for your research on the topic 'Progressive systemic sclerosi.'

Next to every source in the list of references, there is an 'Add to bibliography' button. Press on it, and we will generate automatically the bibliographic reference to the chosen work in the citation style you need: APA, MLA, Harvard, Chicago, Vancouver, etc.

You can also download the full text of the academic publication as pdf and read online its abstract whenever available in the metadata.

Browse journal articles on a wide variety of disciplines and organise your bibliography correctly.

1

Asherson, R. A., H. Angus, J. A. Mathews, O. Meyers, and G. R. Hughes. "The progressive systemic sclerosis/systemic lupus overlap: an unusual clinical progression." Annals of the Rheumatic Diseases 50, no. 5 (May 1, 1991): 323–27. http://dx.doi.org/10.1136/ard.50.5.323.

Full text

APA, Harvard, Vancouver, ISO, and other styles

2

Cascone, Piero, Andrea Rivaroli, and Stefano Vetrano. "Progressive Systemic Sclerosis." Journal of Craniofacial Surgery 9, no. 5 (September 1998): 472–76. http://dx.doi.org/10.1097/00001665-199809000-00018.

Full text

APA, Harvard, Vancouver, ISO, and other styles

3

Lundborg, Christopher N., Petre V. Nitescu, Lennart K. Appelgren, and Ioan D. Curelaru. "Progressive Systemic Sclerosis." Regional Anesthesia and Pain Medicine 24, no. 1 (January 1999): 89–93. http://dx.doi.org/10.1097/00115550-199924010-00016.

Full text

APA, Harvard, Vancouver, ISO, and other styles

4

Roberts, James G., Raj Sabar, John A. Gianoli, and Alan D. Kaye. "Progressive Systemic Sclerosis." Journal of Clinical Anesthesia 14, no. 6 (September 2002): 474–77. http://dx.doi.org/10.1016/s0952-8180(02)00380-x.

Full text

APA, Harvard, Vancouver, ISO, and other styles

5

Fogo, Agnes. "Progressive Systemic Sclerosis." American Journal of Kidney Diseases 34, no. 2 (August 1999): E4—E5. http://dx.doi.org/10.1053/s0272-6386(13)90019-6.

Full text

APA, Harvard, Vancouver, ISO, and other styles

6

Ananyeva, L. P., I. E. Tyurin, O. A. Koneva, L. A. Garzanova, and A. M. Lila. "Interstitial lung disease in systemic sclerosis (systemic scleroderma)." Modern Rheumatology Journal 15, no. 1S (March 17, 2021): 1–62. http://dx.doi.org/10.14412/1996-7012-2021-1s-1-62.

Full text

Abstract:

In systemic sclerosis (SSc), interstitial lung disease (ILD) is common (>80%) and worsens the prognosis of the disease, but severe progressive damage develops only in 8–10% of cases. Interstitial changes in the lungs occur early (within the first 3–5 years of the disease). The histological manifestations are similar to those of idiopathic ILD.The main tool for screening and diagnosing of ILD associated with SSc is high-resolution computed tomography of the lungs, resulting data influence the choice of therapy. In most patients a relatively intact and stable forced vital capacity of the lungs is recorded for a long time, but the diffusion capacity of the lungs decreases early and steadily. Pulmonary functional tests have prognostic value.The choice of the optimal therapy for SSc with lung lesions is based on general disease activity (the severity of inflammation and fibrosis) and the its severity, rate of progression of the disease in general and the leading pathology – interstitial pneumonia (IP) – in particular. In patients with SSc and severe or progressive IP, treatment with mycophenolate mofetil (MMF), cyclophosphamide, nintedanib, or nintedanib in combination with MMF if appropriate, should be considered. If this therapy is ineffective, rituximab may be used.

APA, Harvard, Vancouver, ISO, and other styles

7

Evans, D. J., S. J. Cashman, and M. Walport. "PROGRESSIVE SYSTEMIC SCLEROSIS: AUTOIMMUNE ARTERIOPATHY." Lancet 329, no. 8531 (February 1987): 480–82. http://dx.doi.org/10.1016/s0140-6736(87)92091-5.

Full text

APA, Harvard, Vancouver, ISO, and other styles

8

Haustein, U. F., K. Herrmann, and H. J. Böhme. "Pathogenesis of Progressive Systemic Sclerosis." International Journal of Dermatology 25, no. 5 (June 1986): 286–94. http://dx.doi.org/10.1111/j.1365-4362.1986.tb02244.x.

Full text

APA, Harvard, Vancouver, ISO, and other styles

9

Bollinger, Alfred. "Microangiopathy of Progressive Systemic Sclerosis." Archives of Internal Medicine 146, no. 8 (August 1, 1986): 1541. http://dx.doi.org/10.1001/archinte.1986.00360200103017.

Full text

APA, Harvard, Vancouver, ISO, and other styles

10

Volkmann, Elizabeth R. "Natural history of systemic sclerosis–related interstitial lung disease: How to identify a progressive fibrosing phenotype." Journal of Scleroderma and Related Disorders 5, no. 2_suppl (December 5, 2019): 31–40. http://dx.doi.org/10.1177/2397198319889549.

Full text

Abstract:

The natural history of interstitial lung disease in patients with systemic sclerosis is highly variable. Historical observational studies have demonstrated that the greatest decline in lung function in systemic sclerosis occurs early in the course of the disease; however, not all patients experience a decline in lung function even in the absence of treatment. Furthermore, among patients who do experience a decline in lung function, the rate of decline can be either rapid or slow. The most common clinical phenotypes of systemic sclerosis–related interstitial lung disease are, therefore, as follows: (1) rapid progressors, (2) gradual progressors, (3) stabilizers, and (4) improvers. This review summarizes the features of systemic sclerosis–related interstitial lung disease patients who are more likely to experience rapid progression of interstitial lung disease, as well as those who are more likely not to experience interstitial lung disease progression. Understanding the clinical, biological, and radiographic factors that consistently predict interstitial lung disease–related outcomes in systemic sclerosis is central to our ability to recognize those patients who are at heightened risk for interstitial lung disease progression. With new options available for treating patients with systemic sclerosis–related interstitial lung disease, it is more important than ever to accurately identify patients who may derive the most benefit from aggressive systemic sclerosis–related interstitial lung disease therapy. Early therapeutic intervention in patients with this progressive fibrosing phenotype may ultimately improve morbidity and mortality outcomes in patients with systemic sclerosis–related interstitial lung disease.

APA, Harvard, Vancouver, ISO, and other styles

11

Jin, Tae Sun, Myoung Suk Park, Do Yung Shin, Youn Bok Jang, and Duk Jae Shun. "Bronchioloalveolar Carcinoma in Progressive Systemic Sclerosis." Korean Journal of Internal Medicine 2, no. 1 (January 31, 1987): 52–56. http://dx.doi.org/10.3904/kjim.1987.2.1.52.

Full text

APA, Harvard, Vancouver, ISO, and other styles

12

De, ArunK, Kallol Das, Archan Sil, and Swarnali Joardar. "Progressive systemic sclerosis in a child." Indian Journal of Dermatology 58, no. 5 (2013): 406. http://dx.doi.org/10.4103/0019-5154.117333.

Full text

APA, Harvard, Vancouver, ISO, and other styles

13

Geller, Stephen A., and Fernando P. F. de Campos. "Esophageal involvement in progressive systemic sclerosis." Autopsy and Case Reports 3, no. 3 (2013): 77–79. http://dx.doi.org/10.4322/acr.2013.031.

Full text

APA, Harvard, Vancouver, ISO, and other styles

14

KAUFMANN, JULIE. "Progressive Systemic Sclerosis and Meralgia Paraesthetica." Annals of Internal Medicine 105, no. 6 (December 1, 1986): 973. http://dx.doi.org/10.7326/0003-4819-105-6-973_2.

Full text

APA, Harvard, Vancouver, ISO, and other styles

15

Freitas, J. P., P. Filipe, I. Emerit, P. Meunier, C. F. Manso, and Guerra Rodrigo. "Hyaluronic Acid in Progressive Systemic Sclerosis." Dermatology 192, no. 1 (1996): 46–49. http://dx.doi.org/10.1159/000246314.

Full text

APA, Harvard, Vancouver, ISO, and other styles

16

Shiraishi, Hiroyasu, Junzo Koizumi, Toshihito Suzuki, Katsuyoshi Mizukami, Yoshiro Tanaka, and Toshihiro Sato. "Progressive Systemic Sclerosis with Mental Disorder." Psychiatry and Clinical Neurosciences 45, no. 4 (December 1991): 855–60. http://dx.doi.org/10.1111/j.1440-1819.1991.tb00524.x.

Full text

APA, Harvard, Vancouver, ISO, and other styles

17

Sluis-Cremer, G. K., P. A. Hessel, E. H. Nizdo, A. R. Churchill, and E. A. Zeiss. "Silica, silicosis, and progressive systemic sclerosis." Occupational and Environmental Medicine 42, no. 12 (December 1, 1985): 838–43. http://dx.doi.org/10.1136/oem.42.12.838.

Full text

APA, Harvard, Vancouver, ISO, and other styles

18

Marycz, Thomas, Steffen M. Muehldorfer, Matthias S. Gruschwitz, Alexander Katalinic, Christoph Herold, Christian Ell, and Eckhart G. Hahn. "Gastric involvement in progressive systemic sclerosis." European Journal of Gastroenterology & Hepatology 11, no. 10 (October 1999): 1151–56. http://dx.doi.org/10.1097/00042737-199910000-00013.

Full text

APA, Harvard, Vancouver, ISO, and other styles

19

Veeraraghavan, Srihari, and Om P. Sharma. "Progressive systemic sclerosis and the lung." Current Opinion in Pulmonary Medicine 4, no. 5 (September 1998): 305–9. http://dx.doi.org/10.1097/00063198-199809000-00011.

Full text

APA, Harvard, Vancouver, ISO, and other styles

20

Jharap, B., L. Koudstaal, E. Neefjes-Borst, and S. Van Weyenberg. "Colonic telangiectasias in progressive systemic sclerosis." Endoscopy 44, S 02 (March 6, 2012): E42—E43. http://dx.doi.org/10.1055/s-0031-1291521.

Full text

APA, Harvard, Vancouver, ISO, and other styles

21

Grosskopf, I., J. Streifler, M. Garty, and J. B. Rosenfeld. "Esophageal Bezoar in Progressive Systemic Sclerosis." Journal of Clinical Gastroenterology 8, no. 6 (December 1986): 695. http://dx.doi.org/10.1097/00004836-198612000-00025.

Full text

APA, Harvard, Vancouver, ISO, and other styles

22

Senaldi, G., D. Vergani, A. Mcwhirter, and C. M. Black. "COMPLEMENT ACTIVATION IN PROGRESSIVE SYSTEMIC SCLEROSIS." Lancet 329, no. 8542 (May 1987): 1143–44. http://dx.doi.org/10.1016/s0140-6736(87)91698-9.

Full text

APA, Harvard, Vancouver, ISO, and other styles

23

Woscoff, Alberto, Graciela Remondino, Léon Jaimovich, Gabriela Berengust, and Amanda Rios. "Progressive systemic sclerosis and pemphigus vulgaris." Journal of the American Academy of Dermatology 21, no. 1 (July 1989): 142–44. http://dx.doi.org/10.1016/s0190-9622(89)80358-5.

Full text

APA, Harvard, Vancouver, ISO, and other styles

24

Mishra, Devesh, Gurmohan Singh, and S. K. Gupta. "Progressive Systemic Sclerosis in a Child." International Journal of Dermatology 29, no. 2 (March 1990): 147–48. http://dx.doi.org/10.1111/j.1365-4362.1990.tb04091.x.

Full text

APA, Harvard, Vancouver, ISO, and other styles

25

SPALTRO, FRANCIS X., CAROLYN COTTRILL, CAROL CAHILL, EILEEN DEGNAN, GREGORY J. MULFORD, DWIGHT SCARBOROUGH, ANDREW J. FRANKS, ALBERT S. KLAINER, and EMIL BISACCIA. "EXTRACORPOREAL PHOTOCHEMOTHERAPY IN PROGRESSIVE SYSTEMIC SCLEROSIS." International Journal of Dermatology 32, no. 6 (June 1993): 417–21. http://dx.doi.org/10.1111/j.1365-4362.1993.tb02811.x.

Full text

APA, Harvard, Vancouver, ISO, and other styles

26

Maeda, M., K. Matubara, H. Hirano, H. Watabe, Y. Ichiki, and S. Mori. "Pitting Scars in Progressive Systemic Sclerosis." Dermatology 187, no. 2 (1993): 104–8. http://dx.doi.org/10.1159/000247216.

Full text

APA, Harvard, Vancouver, ISO, and other styles

27

Lock, G., M. Zeuner, M. Kammeri, A. Holstege, B. Lang, and J. Schölmerich. "Gallbladder contractility in progressive systemic sclerosis." Gastroenterology 108, no. 4 (April 1995): A424. http://dx.doi.org/10.1016/0016-5085(95)26012-9.

Full text

APA, Harvard, Vancouver, ISO, and other styles

28

Lucivero, V., D. M. Mezzapesa, M. Petruzzellis, A. Carella, P. Lamberti, and F. Federico. "Ischaemic stroke in progressive systemic sclerosis." Neurological Sciences 25, no. 4 (October 2004): 230–33. http://dx.doi.org/10.1007/s10072-004-0327-z.

Full text

APA, Harvard, Vancouver, ISO, and other styles

29

Herrmann, K., J. Schaller, U. F. Haustein, Ch Baldauf, and St Kie�ig. "Lymphocytotoxic autoantibodies in progressive systemic sclerosis." Archives of Dermatological Research 280, no. 7 (November 1988): 399–404. http://dx.doi.org/10.1007/bf00429977.

Full text

APA, Harvard, Vancouver, ISO, and other styles

30

Oriente, P., R. Scarpa, C. Biondi, A. Riccio, C. Farinaro, and A. Del Puente. "Progressive systemic sclerosis and S-adenosylmethionine." Clinical Rheumatology 4, no. 3 (September 1985): 360–61. http://dx.doi.org/10.1007/bf02031624.

Full text

APA, Harvard, Vancouver, ISO, and other styles

31

Yocum, David E., and Ronald L. Wilder. "Cyclosporin A in progressive systemic sclerosis." American Journal of Medicine 83, no. 2 (August 1987): 369–70. http://dx.doi.org/10.1016/0002-9343(87)90724-8.

Full text

APA, Harvard, Vancouver, ISO, and other styles

32

McAleer, J. J., S. R. Cunningham, W. Dickey, D. Burrows, and M. E. Callender. "Transient ascites in progressive systemic sclerosis." BMJ 293, no. 6556 (November 8, 1986): 1211–12. http://dx.doi.org/10.1136/bmj.293.6556.1211.

Full text

APA, Harvard, Vancouver, ISO, and other styles

33

BIRK, M. A., and E. L. ZEUTHEN. "PHRENICUS PALSY IN PROGRESSIVE SYSTEMIC SCLEROSIS." Rheumatology 34, no. 7 (1995): 684–85. http://dx.doi.org/10.1093/rheumatology/34.7.684.

Full text

APA, Harvard, Vancouver, ISO, and other styles

34

Bahmer, F. A. "Isotretinoin therapy for progressive systemic sclerosis." Archives of Dermatology 121, no. 3 (March 1, 1985): 308b—308. http://dx.doi.org/10.1001/archderm.121.3.308b.

Full text

APA, Harvard, Vancouver, ISO, and other styles

35

Averbuch-Heller, L., I. Steiner, and O. Abramsky. "Neurologic Manifestations of Progressive Systemic Sclerosis." Archives of Neurology 49, no. 12 (December 1, 1992): 1292–95. http://dx.doi.org/10.1001/archneur.1992.00530360094024.

Full text

APA, Harvard, Vancouver, ISO, and other styles

36

Brown, Jeffrey J., and M. John Murphy. "Transverse myelopathy in progressive systemic sclerosis." Annals of Neurology 17, no. 6 (June 1985): 615–17. http://dx.doi.org/10.1002/ana.410170617.

Full text

APA, Harvard, Vancouver, ISO, and other styles

37

Bahmer, Friedrich A. "Isotretinoin Therapy for Progressive Systemic Sclerosis." Archives of Dermatology 121, no. 3 (March 1, 1985): 308. http://dx.doi.org/10.1001/archderm.1985.01660030028010.

Full text

APA, Harvard, Vancouver, ISO, and other styles

38

Rossman, Barry, and Adrian W. Orgniotti. "Progressive systemic sclerosis (scleroderma) and impotence." Urology 33, no. 3 (March 1989): 189–92. http://dx.doi.org/10.1016/0090-4295(89)90388-9.

Full text

APA, Harvard, Vancouver, ISO, and other styles

39

Pamuk, G. E., Ö. N. Pamuk, M. R. Altiparmak, M. Tanverdi, N. Uygun, and S. Yurdakul. "Secondary Amyloidosis in Progressive Systemic Sclerosis." Clinical Rheumatology 20, no. 4 (July 2001): 285–87. http://dx.doi.org/10.1007/s100670170048.

Full text

APA, Harvard, Vancouver, ISO, and other styles

40

Chiou, Allen Wen-Hsyang, Jen-Kou Lin, and Feng-Ming Wang. "Anorectal abnormalities in progressive systemic sclerosis." Diseases of the Colon & Rectum 32, no. 5 (May 1989): 417–21. http://dx.doi.org/10.1007/bf02563695.

Full text

APA, Harvard, Vancouver, ISO, and other styles

41

Ebert, Ellen C. "Esophageal disease in progressive systemic sclerosis." Current Treatment Options in Gastroenterology 11, no. 1 (January 31, 2008): 64–69. http://dx.doi.org/10.1007/s11938-008-0008-8.

Full text

APA, Harvard, Vancouver, ISO, and other styles

42

Goldberg, Jerry, and Andrea Dlesk. "Nifedipine treatment for progressive systemic sclerosis." Arthritis & Rheumatism 29, no. 8 (August 1986): 1053–54. http://dx.doi.org/10.1002/art.1780290820.

Full text

APA, Harvard, Vancouver, ISO, and other styles

43

Nicholson, Dorothy. "Progressive Systemic Sclerosis and Graves' Disease." Archives of Internal Medicine 146, no. 12 (December 1, 1986): 2350. http://dx.doi.org/10.1001/archinte.1986.00360240064012.

Full text

APA, Harvard, Vancouver, ISO, and other styles

44

Johnson, David A. "Pulmonary Disease in Progressive Systemic Sclerosis." Archives of Internal Medicine 149, no. 3 (March 1, 1989): 589. http://dx.doi.org/10.1001/archinte.1989.00390030075014.

Full text

APA, Harvard, Vancouver, ISO, and other styles

45

Arginteanu, Marc S., and Noel I. Perin. "Paraspinal calcinosis associated with progressive systemic sclerosis." Journal of Neurosurgery 87, no. 5 (November 1997): 761–63. http://dx.doi.org/10.3171/jns.1997.87.5.0761.

Full text

Abstract:

✓ The authors describe a case of paraspinal calcinosis in a 65-year-old woman with progressive systemic sclerosis. Although calcinosis occurs in up to 27% of cases of progressive systemic sclerosis, symptomatic paraspinal calcinosis is extremely rare. In the case reported here, multiple cervical facet joints were compromised by progressive calcinosis, leading to glacial spinal instability. Internal fixation was indicated to correct the instability and decompress the spinal canal. Medical therapy was instituted to arrest or reverse the ongoing calcinosis.

APA, Harvard, Vancouver, ISO, and other styles

46

Volkmann, Elizabeth R., Donald P. Tashkin, Myung Sim, Grace Hyun Kim, Jonathan Goldin, and Philip J. Clements. "Determining progression of scleroderma-related interstitial lung disease." Journal of Scleroderma and Related Disorders 4, no. 1 (December 17, 2018): 62–70. http://dx.doi.org/10.1177/2397198318816915.

Full text

Abstract:

Interstitial lung disease occurs in the majority of patients with systemic sclerosis. Although interstitial lung disease is the number one cause of death in systemic sclerosis, interstitial lung disease progression rates vary considerably among patients with systemic sclerosis. Some patients with systemic sclerosis–associated interstitial lung disease have sub-clinical disease and may not derive benefit from immunosuppression, while others have a more aggressive interstitial lung disease phenotype. Reliable predictors of interstitial lung disease progression are lacking. The present review describes our current approach to monitoring systemic sclerosis–associated interstitial lung disease progression in clinical practice. To illustrate the marked heterogeneity that exists in interstitial lung disease progression rates in systemic sclerosis, this review presents the individual disease course of five unique patients with systemic sclerosis–associated interstitial lung disease who participated in the Scleroderma Lung Study II. These cases illustrate that treatment response rates vary in systemic sclerosis–associated interstitial lung disease and more research is needed to determine how to predict treatment response in systemic sclerosis–associated interstitial lung disease and to develop personalized treatment approaches for patients with this devastating disease.

APA, Harvard, Vancouver, ISO, and other styles

47

Garty, Ben-Zion, Balu H. Athreya, Robert Wilmott, Nina Scarpa, Robert Doughty, and Steven D. Douglas. "Pulmonary Functions in Children With Progressive Systemic Sclerosis." Pediatrics 88, no. 6 (December 1, 1991): 1161–67. http://dx.doi.org/10.1542/peds.88.6.1161.

Full text

Abstract:

The patterns of pulmonary involvement in 13 children with progressive systemic sclerosis were investigated. Eight patients (61%) had respiratory symptoms or signs and 7 patients (55%) had abnormalities on chest roentgenogram. Twelve patients (92%) had abnormal pulmonary function tests: 7 had restrictive disease, 2 had obstructive disease, 2 had small airway disease, and 1 had an isolated reduction in the diffusing capacity of carbon monoxide. Nine patients had the test performed during the first year of illness, 3 during the second year, and 1 at 5 years. All patients had abnormal pulmonary function tests when first studied. Subsequent pulmonary function tests over a period of 3 to 10 years (mean 6.2 years) showed substantial changes in only 2 patients (1 patient had initial worsening of diffusing capacity of carbon monoxide followed by normalization and another patient showed improvement of obstructive disease). Two patients died during follow-up, 1 of pulmonary hypertension, the other of severe restrictive lung disease and myocardial fibrosis. The major findings of this study were (1) high frequency of pulmonary disease in children with progressive systemic sclerosis, (2) early involvement of the lungs, (3) relatively indolent progression of lung disease, and (4) the prognostic importance of the severity of pulmonary disease. Pulmonary manifestations of progressive systemic sclerosis in children appear to be similar to those of affected adults.

APA, Harvard, Vancouver, ISO, and other styles

48

Wollin, Lutz, Jörg HW Distler, Christopher P. Denton, and Martina Gahlemann. "Rationale for the evaluation of nintedanib as a treatment for systemic sclerosis–associated interstitial lung disease." Journal of Scleroderma and Related Disorders 4, no. 3 (April 21, 2019): 212–18. http://dx.doi.org/10.1177/2397198319841842.

Full text

Abstract:

Interstitial lung disease is a common manifestation of systemic sclerosis. Systemic sclerosis–associated interstitial lung disease is characterized by progressive pulmonary fibrosis and a reduction in pulmonary function. Effective treatments for systemic sclerosis–associated interstitial lung disease are lacking. In addition to clinical similarities, systemic sclerosis–associated interstitial lung disease shows similarities to idiopathic pulmonary fibrosis in the pathophysiology of the underlying fibrotic processes. Idiopathic pulmonary fibrosis and systemic sclerosis–associated interstitial lung disease culminate in a self-sustaining pathway of pulmonary fibrosis in which fibroblasts are activated, myofibroblasts accumulate, and the excessive extracellular matrix is deposited. Nintedanib is a tyrosine kinase inhibitor that has been approved for the treatment of idiopathic pulmonary fibrosis. In patients with idiopathic pulmonary fibrosis, nintedanib slows disease progression by decreasing the rate of lung function decline. In this review, we summarize the antifibrotic, anti-inflammatory, and attenuated vascular remodeling effects of nintedanib demonstrated in in vitro studies and in animal models of aspects of systemic sclerosis. Nintedanib interferes at multiple critical steps in the pathobiology of systemic sclerosis–associated interstitial lung disease, providing a convincing rationale for its investigation as a potential therapy. Finally, we summarize the design of the randomized placebo-controlled SENSCIS® trial that is evaluating the efficacy and safety of nintedanib in patients with systemic sclerosis–associated interstitial lung disease.

APA, Harvard, Vancouver, ISO, and other styles

49

Zulian, Francesco, Roberto Dal Pozzolo, Alessandra Meneghel, Biagio Castaldi, Renzo Marcolongo, Alida Linda Patrizia Caforio, and Giorgia Martini. "Rituximab for rapidly progressive juvenile systemic sclerosis." Rheumatology 59, no. 12 (May 23, 2020): 3793–97. http://dx.doi.org/10.1093/rheumatology/keaa193.

Full text

Abstract:

Abstract Objective Juvenile systemic sclerosis (JSSc) with rapidly progressive course is a life-threatening condition associated with a poor prognosis. Recently, rituximab (RTX) has been shown to be a promising treatment for adult patients with SSc. We present a series of four patients with rapidly progressive JSSc successfully treated with RTX. Methods Clinical, laboratory and functional parameters were collected from four patients with rapidly progressive JSSc treated with RTX for at least 1 year. All patients underwent four yearly courses of i.v. RTX 375 mg/m2 on day 0 and 14, at 3-month intervals. Low dose oral prednisone and MMF were also administered. Data were recorded at baseline and every 6 months and included pulmonary and myocardial function parameters, muscular, vascular and skin changes. The Juvenile Systemic Sclerosis Severity Score (J4S) estimated the overall disease severity over time. Results Four patients (three males, one female), aged 8–17 years, entered the study. Three patients presented with prevalent cardiac involvement, one with severe pulmonary involvement. After 1 year of RTX treatment, all patients showed significant improvement of J4S, Raynaud’s phenomenon and cutaneous involvement. Among those with prevalent cardiac involvement, two showed an improvement of the myocardial function (left ventricular ejection fraction [EF] +37% and +19%, respectively) and in the third arrhythmias disappeared. The patient with severe pulmonary involvement showed a significant improvement of the respiratory function (forced vital capacity +46%, forced expiratory volume in 1 s +33%, diffusing capacity of the lung for carbon monoxide [DLCO] +30%). No major side effects were reported. Conclusions Our data suggest that a combination of RTX and MMF is effective in arresting the rapid progression of JSSc.

APA, Harvard, Vancouver, ISO, and other styles

50

Saketkoo, Lesley Ann, Mary Beth Scholand, Matthew R. Lammi, and Anne-Marie Russell. "Patient-reported outcome measures in systemic sclerosis–related interstitial lung disease for clinical practice and clinical trials." Journal of Scleroderma and Related Disorders 5, no. 2_suppl (March 2020): 48–60. http://dx.doi.org/10.1177/2397198320904178.

Full text

Abstract:

Systemic sclerosis (SSc) is a progressive vasculopathic, fibrosing autoimmune condition, portending significant mortality; wherein interstitial lung disease (ILD) is the leading cause of death. Although lacking a definitive cure, therapeutics for (SSc-ILD) that stave progression exist with further promising primary and adjuvant compounds in development, as well as interventions to reduce symptom burden and increase quality of life. To date, there has been a significant but varied history related to systemic sclerosis–related interstitial lung disease trial design and endpoint designation. This is especially true of endpoints measuring patient-reported perceptions of efficacy and tolerability. This article describes the underpinnings and complexity of the science, methodology, and current state of patient-reported outcome measures used in (SSc-ILD) systemic sclerosis–related interstitial lung disease in clinical practice and trials.

APA, Harvard, Vancouver, ISO, and other styles

We offer discounts on all premium plans for authors whose works are included in thematic literature selections. Contact us to get a unique promo code!