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Journal articles on the topic 'ANCA vasculitis'
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1
Majumdar, Avirup, Virendra Atam, Saurabh Pandey, Prashant Singh, and Himanshu Chauhan. "Digital infarct and mononeuritis in a middle aged female: always suspect Antineutrophil cytoplasmic antibodies associated vasculitis." International Journal of Advances in Medicine 7, no. 4 (March 21, 2020): 707. http://dx.doi.org/10.18203/2349-3933.ijam20201127.
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Vasculitis is a process caused by inflammation of blood vessel walls and results in a variety of disorders. Small-vessel vasculitis (vasculitis involving arteries, venules and capillaries) should be suspected in any patient who presents with a multisystem disease that is not caused by an infectious or malignant process. Testing for Antineutrophil cytoplasmic antibody (ANCA) is the basis of classification of small vessel vasculitis into ANCA associated and non - ANCA associated vasculitis. Apart from cutaneous manifestations like palpable purpura and vasculitic urticaria, digital gangrene in a patient with evidence of mononeuritis multiplex is highly suggestive of ANCA associated vasculitis (AAV). Clinically most of these vasculitides have overlapping clinical presentations and similar treatment. Early diagnosis and rapid initiation of treatment of AAV is recommended rather than ordering for definitive tests (e.g. histopathology or angiograms) since delay in treatment can result in serious end organ damage (pulmonary or renal).
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Radwan, Yasser, Sarah Berini, Floranne Ernste, and Ashima Makol. "Proteinase 3 (PR3)-antineutrophil cytoplasmic antibody (ANCA)-associated vasculitic neuropathy in diffuse cutaneous systemic sclerosis: a rare duo." BMJ Case Reports 12, no. 11 (November 2019): e232987. http://dx.doi.org/10.1136/bcr-2019-232987.
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Systemic sclerosis (SSc) is characterised by non-inflammatory vasculopathy, autoimmunity and widespread fibrosis. While the presence of antineutrophil cytoplasmic antibodies (ANCAs) has been reported in SSc, their association with ANCA-associated vasculitis is exceedingly rare. Myeloperoxidase ANCA is more common than proteinase-3 ANCA, and glomerulonephritis is the most common clinical presentation of ANCA-associated vasculitis in SSc. ANCAs have been associated with the adverse disease outcomes in SSc, including higher mortality per recent reports. A 65-year-old man with diffuse cutaneous SSc for 6 years presented with new-onset peripheral neuropathy. Workup revealed a positive proteinase-3 and cytoplasmic ANCA, and histopathology confirmed an inflammatory vasculitic neuropathy. The patient was successfully treated with rituximab. Our case highlights the importance of checking ANCA in SSc at baseline, given the risk of disease-related complications, even years later. Tissue biopsy is often warranted for confirmation of vasculitis and prompt treatment can optimise long-term outcomes.
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Csernok, Elena. "The Diagnostic and Clinical Utility of Autoantibodies in Systemic Vasculitis." Antibodies 8, no. 2 (May 1, 2019): 31. http://dx.doi.org/10.3390/antib8020031.
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Considerable progress has been made in understanding the role of autoantibodies in systemic vasculitides (SV), and consequently testing for anti-neutrophil cytoplasmic antibodies (ANCA), anti-glomerular basement membrane antibodies (anti-GBM), and anti-C1q antibodies is helpful and necessary in the diagnosis, prognosis, and monitoring of small-vessel vasculitis. ANCA-directed proteinase 3 (PR3-) or myeloperoxidase (MPO-) are sensitive and specific serologic markers for ANCA-associated vasculitides (AAV), anti-GBM antibodies are highly specific for the patients with anti-GBM antibody disease (formerly Goodpasture’s syndrome), and autoantibodies to C1q are characteristic of hypocomlementemic urticarial vasculitis syndrome (HUVS; anti-C1q vasculitis). The results of a current EUVAS study have led to changes in the established strategy for the ANCA testing in small-vessel vasculitis. The revised 2017 international consensus recommendations for ANCA detection support the primary use PR3- and MPO-ANCA immunoassays without the categorical need for additional indirect immunofluorescence (IIF). Interestingly, the presence of PR3- and MPO-ANCA have led to the differentiation of distinct disease phenotype of AAV: PR3-ANCA-associated vasculitis (PR3-AAV), MPO-ANCA-associated vasculitis (MPO-AAV), and ANCA-negative vasculitis. Further studies on the role of these autoantibodies are required to better categorize and manage appropriately the patients with small-vessel vasculitis and to develop more targeted therapy.
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Arnold, S., J. Mahrhold, A. Kerstein-Staehle, E. Csernok, B. Hellmich, N. Venhoff, J. Thiel, et al. "POS0829 SPECTRUM OF ANCA-SPECIFICITIES IN EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS IN A RETROSPECTIVE MULTICENTER STUDY." Annals of the Rheumatic Diseases 81, Suppl 1 (May 23, 2022): 705–6. http://dx.doi.org/10.1136/annrheumdis-2022-eular.3083.
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BackgroundAnti-neutrophil cytoplasmic autoantibodies specific for myeloperoxidase (MPO-ANCA) are found in 10-70% of the patients with eosinophilic granulomatosis with polyangiitis (EGPA) depending on disease activity, methodological aspects and cohort examined [1-3]. Recently, a higher prevalence of anti-pentraxin 3 (PTX3)-ANCA has been reported in EGPA compared to granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) [4].ObjectivesTo investigate the spectrum of ANCA specificities in a multicenter cohort of patients with EGPA and identify novel ANCA antigens.MethodsWe conducted a retrospective analysis of 73 patients with EGPA treated between 2015 and 2020 in 3 tertiary referral centers. In addition to in-house ANCA testing with indirect immunofluorescence (IFT) on fixed human granulocytes and antigen-specific enzyme-linked immunosorbent assays (ELISA), ANCA specificities were determined using a cell-based assay (CBA; Euroimmun, Lübeck, Germany). Diagnosis was based on Chapel Hill consensus conference definitions, ACR- and MIRRA-criteria for EGPA. Patient characteristics and clinical manifestations were evaluated and compared based on ANCA status. Fisher`s exact test was employed for comparison of patient groups.ResultsANCA findings are summarized in Table 1. MPO- and proteinase 3 (PR3)-ANCA positive patients (13.7%) had a higher prevalence of peripheral neuropathy (70% vs. 44.4%, p = 0.0003) and glomerulonephritis (20% vs. 14.3%, not significant). MPO- and PR3-ANCA-negative patients (86.3%) had a higher prevalence of heart (10% vs. 46%, p <0.0001), central nervous system (CNS) (0% vs. 14.3%, p <0.0001) and gastrointestinal (10% vs. 22.2%, p = 0.0327) involvement. PTX3-ANCA were associated with a higher prevalence of ear-nose-throat (ENT) (100% vs. 85.3%, p <0.0001), lung (100% vs. 89.7%, p = 0.0015), gastrointestinal involvement (60% vs. 17.6%, p <0.0001) and peripheral neuropathy (100% vs. 48.5%, p <0.0001). Kidney (0% vs. 16.2%, p <0.0001) and CNS involvement (0% vs. 13.2%, p = 0.0002) occurred less frequently in PTX3-ANCA positive patients. The 2 olfactomedin 4 (OLM4)-ANCA positive patients presented with ENT, lung and kidney involvement, and polyneuropathy, respectively.Table 1.ANCA in EGPA cohort (n = 73). BPI = bactericidal permeability-increasing protein.IFT / ELISANo. of patients (%)P-ANCA11 (15.1)C-ANCA5 (6.8)MPO-ANCA8 (10.9)PR3-ANCA2 (2.7)BPI-ANCA1 (1.4)PTX3-ANCA5 (6.8)OLM4-ANCA2 (2.7)ConclusionWe report on the detection of PTX3-, BPI- and OLM4-ANCA in addition to MPO- and PR3-ANCA in EGPA. OLM4-ANCA has been reported in 2 patients with non-vasculitic inflammatory symptoms previously [5]. Herein, detection of OLM4-ANCA in EGPA is reported for the first time. Our study shows that the presence of ANCA with various specificities other than MPO and PR3 contribute to a higher prevalence of ANCA in EGPA. Moreover, clinical manifestations differ between ANCA-negative EGPA and ANCA-positive EGPA, and between patients with different ANCA-specificities.References[1]Schönermarck U, et al. Prevalence and spectrum of rheumatic diseases associated with proteinase 3-antineutrophil cytoplasmic antibodies (ANCA) and myeloperoxidase-ANCA. Rheumatology 2001;40:178-84.[2]Bremer P, et al. Getting rid of MPO-ANCA: a matter of disease subtype. Rheumatology 2013:752-4.[3]Comarmond C, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis Rheum 2013;65:270-81.[4]Padoan R, et al. IgG anti-Pentraxin 3 antibodies are a novel biomarker of ANCA-associated vasculitis and better identify patients with eosinophilic granulomatosis with polyangiitis. J Autoimmun 2021;124:102725.[5]Amirbeagi F, et al. Olfactomedin-4 autoantibodies give unusual c-ANCA staining patterns with reactivity to a subpopulation of neutrophils. J Leukoc Biol 2015;97:181-9.Disclosure of InterestsNone declared
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Saha, Biplab, Aditi Saha, Fernanda Cordeiro-Rudnisky, Boris Shkolnik, and Scott Beegle. "Destructive Upper Airway Disease from Eosinophilic Granulomatosis with Polyangiitis (EGPA): The Very First Case." Case Reports in Rheumatology 2019 (May 23, 2019): 1–4. http://dx.doi.org/10.1155/2019/6173869.
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Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystem vasculitic disorder that predominantly affects medium- and small-sized blood vessels. EGPA belongs to a group of vasculitides known as anti-neutrophil cytoplasmic antibody- (ANCA-) associated vasculitis (AAV). Upper airway involvement is seen in all ANCA-associated vasculitides, but destructive upper airway disease has never been reported in patients with EGPA. We report the first case of erosive chondritis and saddle nose deformity in a 50-year-old patient suffering from EGPA.
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Segelmark, Mårten. "Serological testing in small vessel vasculitis." Rheumatology 59, Supplement_3 (April 29, 2020): iii51—iii54. http://dx.doi.org/10.1093/rheumatology/kez633.
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Abstract Serological analysis has a central role in the diagnostic work-up of patients with suspected small vessel vasculitis, both for establishing a specific diagnosis and for the monitoring of response to therapy. Autoantibodies can be detected in all forms of primary small vessel vasculitis as well as in the most common forms of secondary vasculitis. For primary vasculitis the most important serological test is for ANCA. ANCA can be found in 75–95% of patients with pauci-immune small vessel vasculitis leading to this subgroup of vasculitides being named ANCA associated vasculitis. ANCA levels often follow this disease course, but the value of serial ANCA testing is controversial. Other important autoantibodies in primary small vessel vasculitis are anti-glomerular basement membrane antibodies, anti-C1q, anti-galactose deficient IgA and cryoglobulins. A wide variety of systemic inflammatory diseases and infections can be complicated by small vessel vasculitis and detected by serological testing. Important examples are SLE, rheumatoid arthritis, Hepatitis C and HIV.
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Preto, Clara, Armandina Silva, Sandra Alves, Margarida Guedes, Paula Matos, Conceição Mota, Paula Rocha, and Paula C. Fernandes. "The Diagnosing Challenge of a Positive ANCA Vasculitis in the Paediatric Age." Case Reports in Pediatrics 2017 (2017): 1–5. http://dx.doi.org/10.1155/2017/2962794.
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ANCA-positive systemic vasculitides, rare in paediatric age, present multiorganic involvement. A female teenager presented with a history of subglottic stenosis diagnosed at the age of 12. From the investigation carried out, we highlight hematoproteinuria and negative ANCAs. At 15 years old, she was admitted for gastrointestinal symptoms and respiratory distress. She presented poor peripheral perfusion, pulmonary haemorrhage, respiratory failure, and severe renal insufficiency. She was started mechanical ventilation and emergency haemodialysis. The immunological study revealed ANCA MPO positive. A presumptive diagnosis of ANCA-positive vasculitis was made, and she was started corticotherapy, cyclophosphamide, and plasmapheresis. A renal biopsy, performed later, showed crescentic glomerulonephritis with chronicity signs. Positive ANCA vasculitis may progress slowly or suddenly. The diagnosis was confirmed by a biopsy; however, we can make a presumptive diagnosis based on clinical findings and in a positive ANCA test in order to start an early treatment and decrease the associated morbimortality.
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Palmucci, Stefano, Corrado Inì, Salvatore Cosentino, Luigi Fanzone, Stefano Di Pietro, Alessia Di Mari, Federica Galioto, et al. "Pulmonary Vasculitides: A Radiological Review Emphasizing Parenchymal HRCT Features." Diagnostics 11, no. 12 (December 9, 2021): 2318. http://dx.doi.org/10.3390/diagnostics11122318.
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Vasculitides represent a heterogeneous group of immune-mediated disorders, characterized by a systemic inflammatory destructive process of the blood vessels resulting either in ischemia or hemorrhage. The organ involved and vessel size influence the pattern of presentation of the pathology. The lung is commonly involved in systemic vasculitides, with heterogeneous clinical, radiological, and histopathological presentations. Primary vasculitides most commonly associated with lung parenchymal involvement include small-vessel antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides, such as granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA). Several studies have reported cases of interstitial lung diseases (ILDs) associated with systemic vasculitis, particularly those positive for ANCA associated vasculitis/vasculitidis: AAV. We have selected from our case series different radiological features of pulmonary vasculitis (i.e., solitary or multiple nodules, cavitary lesions, nodules with centrilobular or peribronchial distribution, airspace consolidations, “crazy paving” appearance, interstitial disease), including cases with interstitial lung alterations. Therefore, the aim of this review is to describe the typical clinical manifestations of vasculitides and their main radiologic features (especially AAV).
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Alba, Marco, J. Jennette, and Ronald Falk. "Pathogenesis of ANCA-Associated Pulmonary Vasculitis." Seminars in Respiratory and Critical Care Medicine 39, no. 04 (August 2018): 413–24. http://dx.doi.org/10.1055/s-0038-1673386.
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AbstractAntineutrophil cytoplasmic antibodies (ANCAs) are autoantibodies specific for antigens located in the cytoplasmic granules of neutrophils and lysosomes of monocytes. ANCAs are associated with a spectrum of necrotizing vasculitis that includes granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis. Pulmonary vasculitis and related extravascular inflammation and fibrosis are frequent components of ANCA vasculitis. In this review, we detail the factors that have been associated with the origin of the ANCA autoimmune response and summarize the most relevant clinical observations, in vitro evidence, and animal studies strongly indicating the pathogenic potential of ANCA. In addition, we describe the putative sequence of pathogenic mechanisms driven by ANCA-induced activation of neutrophils that result in small vessel necrotizing vasculitis and extravascular granulomatous necrotizing inflammation.
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Yoneva, Tzvetelina, Yana Zdravkova, Georgi Kotov, Rasho Rashkov, and Ivan Sheytanov. "Structure of the vasculitides observed in the Clinic of Rheumatology." Revmatologiia (Bulgaria) 28, no. 3 (November 16, 2020): 7–24. http://dx.doi.org/10.35465/28.3.2020.pp7-24.
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Systemic vasculitides are a heterogenic group of disorders characterized by destructive inflammation and fibrinoid necrosis of the vascular wall, vessel occlusion and tissue ischemia. Vasculitides presenting with necrosis are included in neither of the contemporary classifications, even though this type of vascular pathology is the one with the most dramatic manifestations in rheumatology. There have been no analyses of the nosological association, clinical features and therapeutic management of the vasculitides with necrosis in the pertinent literature. The aim of the present study was to analyze vasculitides in the Bulgarian population in terms of their nosological association; to examine the portion that ANCA-associated vasculitides represent out of all vasculitic syndromes on the one hand, and to make an analysis of the vasculitides with necrosis according to their nosological association on the other. In the present study, we included 388 patients with vasculitis, 251 of whom were female and 137 male. We conducted a prospective and retrospective analysis which covered the patients with vasculitis who were admitted to the Clinic of Rheumatology over the period 2009-2018. ANCA-associated vasculitides were the most often diagnosed vasculitides in the Clinic of Rheumatology. Vasculitic manifestations over the course of connective tissue diseases (most often systemic lupus erythematosus and systemic sclerosis) were the second most common group. Life-threatening cases of vasculitis with necrosis were mainly the result of flares of different connective tissue diseases. The major necrotizing vasculitides (Wegener’s granulomatosis and microscopic polyangiitis) were responsible for 12.6% of the cases of vasculitis with necrosis. In order to establish the correct diagnosis and start the suitable treatment, it is of vital importance to recognize the different vasculitic syndromes and their wide differential diagnosis. Most of them respond well to the currently available therapeutic options, especially if the correct diagnosis has been established early.
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Pefanis, A., DS Williams, H. Skrzypek, A. Fung, and K. Paizis. "A case of ANCA-associated vasculitis presenting de novo in pregnancy, successfully treated with rituximab." Obstetric Medicine 13, no. 1 (July 26, 2018): 41–44. http://dx.doi.org/10.1177/1753495x18780853.
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Antineutrophil cytoplasm antibody (ANCA)-associated vasculitides are rare small vessel vasculitides of unknown cause. The pathogenic role of MPO-ANCA in the vasculitides has been supported using various animal models, with B-cells playing a role in the disease pathogenesis. Pregnancy in the presence of an autoimmune disease such as vasculitis is often associated with significant morbidity. Little is known about the outcomes when women present with de novo vasculitis during pregnancy, and the appropriate management of such presentations is unclear. We describe a case of a 33-year-old female presenting in her second pregnancy with new onset ANCA vasculitis at 12 weeks’ gestation. She was successfully treated with prednisolone and rituximab, and delivered a healthy 2.8 kg boy at 36 weeks’ gestation with no clinical manifestations of vasculitis or neutropenia in the neonate.
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Litvinova, M., N. Bulanov, P. Novikov, N. Vlasenko, E. Filatova, K. Kurginyan, A. Skvortsov, and S. Moiseev. "Granulomatosis with polyangiitis and microscopic polyangiitis: common features and differences." Clinical pharmacology and therapy 36, no. 1 (February 27, 2023): 64–72. http://dx.doi.org/10.32756/0869-5490-2023-1-64-72.
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Patients with ANCA-associated vasculitides usually present with similar clinical manifestations (fever, joint pain, lung and kidney diseases, purpura, etc.). However, signs of granulomatosis inflammation of the upper and lower respiratory tract and/or orbit allow to differentiate granulomatosis with polyangiitis (GPA) from microscopic polyangiitis (MPA). Circulating ANCAs are the key laboratory sign of ANCA-associated vasculitis, although they can be negative in a proportion of patients and can be found in patients with other diseases. GPA is usually associated with ANCAs againts proteinase-3, whereas ANCAs against myeloperoxidase are more common in patients with MPA. The authors present two patients with ANCA-associated vasculitides and discuss the common clinical features and differences between GPA and MPA and new classification criteria.
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Jennette, J. Charles, and Ronald J. Falk. "ANCA Vasculitis." Pathology Case Reviews 12, no. 5 (September 2007): 200–204. http://dx.doi.org/10.1097/pcr.0b013e3181557f6e.
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Hilhorst, Marc, Pieter van Paassen, and Jan Willem Cohen Tervaert. "Proteinase 3-ANCA Vasculitis versus Myeloperoxidase-ANCA Vasculitis." Journal of the American Society of Nephrology 26, no. 10 (May 8, 2015): 2314–27. http://dx.doi.org/10.1681/asn.2014090903.
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Calhan, Turan, Abdurrahman Sahin, Resul Kahraman, Mustafa Erhan Altunoz, Fatma Ozbakır, Kamil Ozdil, and Hacı Mehmet Sokmen. "Antineutrophil Cytoplasmic Antibody Frequency in Chronic Hepatitis B Patients." Disease Markers 2014 (2014): 1–5. http://dx.doi.org/10.1155/2014/982150.
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Background. Chronic hepatitis B (CHB) is a viral disease, common across the world, and associated with several extraintestinal manifestations including vasculitis. Antineutrophil cytoplasmic antibodies (ANCAs) are sensitive and specific markers for vasculitides. There is limited data available in the literature on whether ANCA formation is stimulated by CHB infection. In the present study we aimed to identify the frequency of ANCA in CHB patients.Methods. A total of 174 subjects were included in the study (87 CHB patients, 87 control subjects). Perinuclear-ANCA (P-ANCA), cytoplasmic-ANCA (C-ANCA), myeloperoxidase ANCA (MPO-ANCA), and proteinase 3-ANCA (PR3-ANCA) were studied. IFA was used for P-ANCA and C-ANCA assays, and ELISA was used for MPO-ANCA and PR3-ANCA assays.Results. ANCA positivity was high in both groups (31% in the CHB group and 26% among controls). There were no significant differences between the groups for P-ANCA and MPO-ANCA (P= 0.6 andP= 0.6, resp.). Frequency of borderline positive C-ANCA and all positive PR3-ANCA (positive + borderline positive) was significantly higher in the CHB group (P= 0.009 andP= 0.005, resp.).Conclusions. In the present study, the frequency of ANCA was high in both groups. The CHB group had a relatively higher frequency of ANCA positivity compared to controls. Borderline positive C-ANCA and positive PR3-ANCA were significantly higher in the CHB group. These results suggest that ANCA may have a high prevalence in Turkey. Patients with CHB should be evaluated particularly for C-ANCA and PR3-ANCA in the presence of vasculitic complaints and lesions.
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Savenkova, N. D. "Treatment strategy ANCA-associated renal vasculitides in children and adolescents." Nephrology (Saint-Petersburg) 23, no. 5 (August 8, 2019): 107–15. http://dx.doi.org/10.24884/1561-6274-2019-23-5-107-115.
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The article presents the terminology and classification in accordance with the International Сhapel Hill Сonsensus Сonference nomenclature of vasculitides (2012), clinical, immunological and morphological manifestations, therapy strategy and outcome of Antineutrophil cytoplasmic antibody vasculitides (ANCA) renal associated vasculitis (microscopic polyanghiitis, granulomatosis with Wegener's polyangiitis, eosinophilic granulomatosis with polyangiitis Churg-Strauss) in children and adolescents. IgG class antibodies to MPO and PR3, histopathological changes in renal biopsy specimens are considered the gold standard in the diagnosis of ANCA-glomerulonephritis. Following the recommendations of The European Vasculitis Study Group (EUVAS) in adult patients, ANCA-associated vasculitis describes the categories of disease severity: localized, early systemic, severe, generalized, refractory. An algorithm for the treatment of ANCA-associated vasculitis, recommended by EULAR (2009) for adult patients and adapted for children of L.A. Plumb et al (2018), which provides for a differentiated approach to the induction of remission in localized, early systemic, severe, generalized, refractory categories of severity and supportive therapy in localized, early systemic, generalized categories, second-line therapy. In most cases of ANCA-associated renal vasculitis in children and adolescents, it is kidney damage that manifests rapidly progressive glomerulonephritis with acute kidney damage, determines the severity and prognosis of outcome in terminal uremia. It seems important and necessary in the treatment protocols of ANCA-associated vasculitis to include a strategy for pre-dialysis and dialysis of rapidly progressive glomerulonephritis with acute kidney damage in children and adolescents.
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Haubitz. "ANCA-associated vasculitis: Diagnosis, clinical characteristics and treatment." Vasa 36, no. 2 (May 1, 2007): 81–89. http://dx.doi.org/10.1024/0301-1526.36.2.81.
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The primary systemic vasculitides are a group of diseases characterized by an inflammatory process of the vessel walls and classified according to the smallest vessels involved. Small vessel vasculitides comprise the largest subgroup divided into diseases with a pauci-immune vasculitis and ANCA and diseases with deposition of immunoglobulin without ANCA. ANCA-associated systemic vasculitides include Wegener’s granulomatosis, microscopic polyangiitis comprising renal-limited vasculitis and Churg-Strauss syndrome. Diagnosis is based on clinical manifestation, ANCA-testing and histology. Beside the role of ANCA as a diagnostic marker many studies and animal models have focused on the pathogenic role. The treatment of ANCA-associated vasculitis has changed from a standardized "Fauci-protocol" to an individualized less toxic strategy taking into consideration disease severity, organ manifestation, age of the patient and individual risk factors (e.g. increased bone marrow toxicity in patients with renal insufficiency). For remission induction patients are sub-grouped according to limited or generalized disease with moderate or severe renal involvement. Thus cyclophosphamide is only used in patients with generalized disease or – regarding Churg-Strauss-syndrome – patients with risk factors. For maintenance of remission azathioprine should be used in most of the patients.
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Abdal, Syed Jamil, Md Zilan Miah Sarker, Syed Md Monowar Ali, A. K. M. Motiur Rahman Bhuiyan, and Md Nahiduzzaman Shazzad. "ANCA-negative Churg-Strauss Syndrome." Bangabandhu Sheikh Mujib Medical University Journal 6, no. 2 (August 4, 2016): 178. http://dx.doi.org/10.3329/bsmmuj.v6i2.29139.
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A rare and a disease of unknown etiology, Churg-Strauss syndrome (CSS) is a granulomatous necrotizing small vessel vasculitis characterized by the presence of asthma, sinusitis, and hypereosinophilia, which is initially described by Churg and Strauss in 1951. Because of its clinical and pathological features that overlap with those of the other anti-neutrophil antibody (ANCA)-associated systemic vasculitides (AASVs) and now the disease is classified as AASVs. The ANCA status may dictate the clinical phenotype. ANCA-positive patients are significantly more likely to have disease manifestations associated with small-vessel vasculitis, including oecrotising glomemlonephritis, mononeuritis and purpura, whereas ANCA-negative cases predominantly likely to have cardiac and lung involvement. The objective of this case report is to point out the possibility of vasculitic rash in ANCA-negative CSS in a 35-year-old man and the disease rarely occurs in Bangladeshi population. We analyze the history, clinical examinations and relevant investigations related to the patient to establish the diagnosis in our department. The clinical scenario and biopsy help us to attain the diagnosis. But due to unavailability of patients' cohort we have limitations of comparison of ANCA status in Bangladeshi populations. Though ANCA-positive and ANCA-negative CSS differ phenotypically, primary therapy for both the conditions is systemic glucocorticoids. Additional immunosuppressive agents like cyclophosphamide, mycophenolate mofetil, azathioprine, rituxin1ab are occasionally added in patients with more advanced or refractory disease.
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Kao, Lily, and Cornelia Weyand. "Vasculitis in Systemic Sclerosis." International Journal of Rheumatology 2010 (2010): 1–9. http://dx.doi.org/10.1155/2010/385938.
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Systemic sclerosis (SSc) is a multiorgan connective tissue disease characterized by autoantibody production and fibroproliferative stenosis of the microvasculature. The vascoluopathy associated with SSc is considered to be noninflammatory, yet frank vasculitis can complicate SSc, posing diagnostic and therapeutic challenges. Here, we have reviewed the literature for reports of small-, medium-, and large-vessel vasculitis occurring in SSc. Amongst 88 reported cases of vasculitis in SSc, patients with ANCA-associated vasculitis appear to present a unique subclass in that they combined typical features of SSc with the renal manifestation of ANCA-associated glomerulonephritis. Other vasculitic syndromes, including large-vessel vasculitis, Behcet's disease, cryoglobulinemia, and polyarteritis nodosa, are rarely encountered in SSc patients. ANCA-associated vasculitis needs to be considered as a differential diagnosis in SSc patients presenting with renal insufficiency, as renal manifestations may result from distinct disease processes and require appropriate diagnostic testing and treatment.
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Sinclair, David, and Judith M. Stevens. "Role of antineutrophil cytoplasmic antibodies and glomerular basement membrane antibodies in the diagnosis and monitoring of systemic vasculitides." Annals of Clinical Biochemistry: International Journal of Laboratory Medicine 44, no. 5 (September 1, 2007): 432–42. http://dx.doi.org/10.1258/000456307781646049.
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Systemic vasculitis, although rare, is often diagnosed late and long after the onset of symptoms. The small vessel vasculitides are recognized clinically by their multisystem presentation, markers of inflammation and evidence for an acute glomerulonephritis (GN), with the most apparent organ involved directing referral to secondary care. Routine laboratory tests are usually non-specific in systemic vasculitis but the use of anti-neutrophil cytoplasmic antibodies (ANCAs) and glomerular basement membrane (GBM) antibodies can aid diagnosis, treatment and monitoring decisions. These antibodies are detected and quantified by indirect immunofluorescence (IIF) and antigen-specific enzyme-linked immunosorbent assay (ELISA), usually in combination for ANCA, and ELISA systems (or direct IIF on kidney biopsy) for GBM antibodies. The presence or absence of ANCA does not confirm or exclude the diagnosis of systemic vasculitis but negative and positive predictive values will be strongly influenced by clinical presentation. Various large studies have been unable to conclude that following serial ANCA titres has great clinical utility in each case but each patient must be considered on its own merits; for example the reappearance of ANCA in a patient who was rendered ANCA negative following treatment is more likely to indicate relapse. The adoption of consensus guidelines that direct testing towards patients with rapidly progressive GN, pulmonary haemorrhage, persistent and destructive ear, nose and upper airways problems, such as subglottic tracheal stenosis, a retro-orbital mass and cutaneous vasculitis with systemic features or peripheral neuropathy, will greatly increase the clinical utility and positive predictive value of these tests.
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Ko, Jihee, and Jay Chol Choi. "Rapid progression of large intracranial cerebral artery involvement in a patient with myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitis." Journal of Medicine and Life Science 21, no. 1 (January 31, 2024): 15–19. http://dx.doi.org/10.22730/jmls.2024.21.1.15.
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Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic necrotizing vasculitis that predominantly affects small vessels of the body. The two most common ANCAs are myeloperoxidase ANCA and proteinase 3 ANCA. Neurological manifestations are frequent in patients with AAV, including peripheral neuropathy, meningitis, and stroke. AAV-associated ischemic stroke usually affects small vessels supplying the white matter or brainstem. This case report details the presentation and treatment course of a 70-year-old man with rapidly progressive multiple intracranial large artery involvement attributed to myeloperoxidase ANCA-associated vasculitis. Despite treatment with high-dose steroids and a rituximab infusion, the patient developed new speech difficulties and respiratory distress, and brain imaging confirmed new stroke lesions with progressive multiple intracranial large cerebral artery involvement. The patient died from SARS-CoV-2 infection 4 months after the diagnosis. This case emphasized the rare presentation of rapidly progressive large vessel involvement in a patient with myeloperoxidase ANCA-associated vasculitis despite active immunotherapy.
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Rao, Deepak A., Kevin Wei, Joseph F. Merola, William R. O’Brien, Samuel U. Takvorian, Paul F. Dellaripa, and Peter H. Schur. "Myeloperoxidase-antineutrophil Cytoplasmic Antibodies (MPO-ANCA) and Proteinase 3-ANCA without Immunofluorescent ANCA Found by Routine Clinical Testing." Journal of Rheumatology 42, no. 5 (April 1, 2015): 847–52. http://dx.doi.org/10.3899/jrheum.140941.
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Objective.Concurrent testing for serum antineutrophil cytoplasmic antibodies (ANCA) by indirect immunofluorescence (IF) and by antiproteinase 3 (PR3)/antimyeloperoxidase (MPO) antibody assays may identify patients with PR3-ANCA or MPO-ANCA despite a negative IF (IF-negative MPO/PR3-positive); however, the significance of this result is not clear. We sought to determine whether IF-negative, MPO/PR3-positive results identified any cases of clinically meaningful systemic vasculitis at our institution.Methods.We conducted a retrospective chart review of all IF-negative, MPO/PR3-positive patients identified at our institution over a 2-year period.Results.Of the 2345 samples tested over 2 years, 1998 were IF-negative. Among these IF-negative samples, 49 samples (2.5%) derived from 38 patients tested positive for MPO-ANCA or PR3-ANCA. Only 1 IF-negative, MPO/PR3-positive patient was subsequently diagnosed with ANCA-associated vasculitis (AAV). Eleven IF-negative, MPO/PR3-positive patients (29%) had been previously diagnosed and treated for AAV, all with positive IF and antibody tests prior to treatment. Four patients had evidence of cutaneous vasculitis not attributed to AAV, while several of the remaining IF-negative, MPO/PR3-positive patients had other immunologic disorders, including systemic lupus erythematosus (5 patients) and inflammatory bowel disease (3 patients).Conclusion.In this real-life cohort assayed simultaneously by IF and multiplexed bead assays, the detection of MPO-ANCA or PR3-ANCA without a positive IF rarely led to a new diagnosis of systemic vasculitis, and was more likely to occur in the context of a non-vasculitic inflammatory condition. Our results suggest that concurrent IF and MPO/PR3 testing may be of limited use in preventing a missed diagnosis of new-onset AAV.
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Kawakami, T., Y. Soma, C. Saito, H. Ogawa, Y. Nagahuchi, T. Okazaki, S. Ozaki, and M. Mizoguchi. "Cutaneous manifestations in patients with microscopic polyangiitis: two case reports and a minireview." Acta Dermato-Venereologica 86, no. 2 (May 2, 2006): 144–47. http://dx.doi.org/10.2340/00015555-0034.
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Microscopic polyangiitis is a systemic small vessel vasculitis, which often has cutaneous and musculoskeletal features. Microscopic polyangiitis is a member of the family of anti-neutrophil cytoplasmic auto-antibody (ANCA)-associated vasculitides and is strongly associated with anti-myeloperoxidase (MPO)-ANCA. Titres of MPO-ANCA may reflect disease activity and play a pathogenic role. Patients with microscopic polyangiitis usually present with erythematous macules on the extremities as the first cutaneous manifestation. Skin biopsy specimens from the erythema reveal small-sized vessels that are infiltrated with neutrophils, consistent with leukocytoclastic vasculitis, in the deep dermis to the subcutaneous fat tissue. The cutaneous involvement is present at an early stage of microscopic polyangiitis with other non-specific symptoms, such as arthralgias and myalgias. The initial cutaneous manifestations are important in early diagnosis of possible ANCA-associated vasculitides with elevated ANCA titres.
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Takahashi, Mie, Haruki Koike, Shohei Ikeda, Yuichi Kawagashira, Masahiro Iijima, Atsushi Hashizume, Masahisa Katsuno, and Gen Sobue. "Distinct pathogenesis in nonsystemic vasculitic neuropathy and microscopic polyangiitis." Neurology - Neuroimmunology Neuroinflammation 4, no. 6 (October 23, 2017): e407. http://dx.doi.org/10.1212/nxi.0000000000000407.
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Objective:To investigate the mechanisms of vasculitis in nonsystemic vasculitic neuropathy (NSVN) and microscopic polyangiitis (MPA), focusing on complement- and antineutrophil cytoplasmic antibody (ANCA)-associated pathogenesis.Methods:Sural nerve biopsy specimens taken from twenty-four patients with NSVN and 37 with MPA-associated neuropathy (MPAN) were examined. Twenty-two patients in the MPAN group tested positive for ANCA.Results:Immunostaining for complement component C3d deposition showed more frequent positive staining of epineurial small vessels in NSVN than in MPAN (p = 0.002). The percentages of C3d-positive blood vessels were higher in the NSVN group than those in the ANCA-positive MPAN and ANCA-negative MPAN groups (p = 0.002 and p = 0.009, respectively). Attachment of neutrophils to the endothelial cells of epineurial small vessels was frequently observed in the MPAN groups, irrespective of the presence or absence of ANCA, but was scarce in the NSVN group. Immunohistochemistry using antimyeloperoxidase (MPO) antibodies revealed that the number of MPO-positive cells attached to the endothelial cells of epineurial vessels was lower in the NSVN group than that in the ANCA-positive MPAN and ANCA-negative MPAN groups (p < 0.001 and p = 0.011, respectively).Conclusions:NSVN and MPA have distinct mechanisms of vasculitis. In MPA, the attachment of neutrophils to vascular endothelial cells seems to be an initial lesion of vasculitis, regardless of the presence or absence of ANCA. Complement participated in the pathogenesis of vasculitis in NSVN.
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Vandergheynst, F. "ANCA-ASSOCIATED VASCULITIS." Acta Clinica Belgica 68, no. 6 (November 2013): 406–10. http://dx.doi.org/10.2143/acb.3436.
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Yates, Max, and Richard Watts. "ANCA-associated vasculitis." Clinical Medicine 17, no. 1 (February 2017): 60–64. http://dx.doi.org/10.7861/clinmedicine.17-1-60.
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Kazachkina, E. O., A. V. Liugai, M. R. Khommyatov, and K. O. Voznyuk. "ANCA-ASSOCIATED VASCULITIS." Journal of scientific articles Health and Education millennium 20, no. 9 (September 30, 2018): 92–95. http://dx.doi.org/10.26787/nydha-2226-7425-2018-20-9-92-95.
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Sharma, Purva, Reza Zonozi, and Duvuru Geetha. "ANCA-Associated Vasculitis." Advances in Kidney Disease and Health 31, no. 3 (May 2024): 194–205. http://dx.doi.org/10.1053/j.akdh.2024.04.005.
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Salvador, Fernando. "ANCA associated vasculitis." European Journal of Internal Medicine 74 (April 2020): 18–28. http://dx.doi.org/10.1016/j.ejim.2020.01.011.
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Uhm, Wan-sik. "ANCA Associated Vasculitis." Journal of the Korean Rheumatism Association 17, no. 2 (2010): 108. http://dx.doi.org/10.4078/jkra.2010.17.2.108.
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Kamesh, L. "ANCA-Positive Vasculitis." Journal of the American Society of Nephrology 13, no. 7 (July 1, 2002): 1953–60. http://dx.doi.org/10.1097/01.asn.0000016442.33680.3e.
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Cooke, Hannah, Matthew Wells, Keith Miller, Andrew R. L. Medford, and Sam Patel. "ANCA-negative ANCA-associated vasculitis: pitfalls of the ‘vasculitis screen’." Clinical Medicine 23, no. 6 (November 2023): 630–32. http://dx.doi.org/10.7861/clinmed.2023-0398.
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Hurtado, Maite, Kateir Contreras, Camilo Alberto González, and Alvaro Bustamante. "Vasculitis ANCA and Systemic Scleroderma." Revista Colombiana de Nefrología 3, no. 2 (July 1, 2016): 137–42. http://dx.doi.org/10.22265/acnef.3.2.220.
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Du, Jie, Hongyue Wang, Lili Zhang, Hongyu Li, Shuang Li, Chao Zhang, Fangfang Sun, and Lirong Zhao. "Axillary lymphadenopathy as the initial manifestation in ANCA-associated systemic vasculitis: A case report." Medicine 102, no. 29 (July 21, 2023): e34218. http://dx.doi.org/10.1097/md.0000000000034218.
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Background: The anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitides are a collection of relatively rare autoimmune diseases characterized by the presence of ANCAs, predominantly against myeloperoxidase and proteinase 3. Multiple organs and systems are involved, but superficial lymph node involvement is rarely reported. Case presentation: A 31-year-old woman initially presented with bilateral axillary lymphadenopathy and that the hilums were not clear. We report the rare case of a patient who presented with an ANCA-associated systemic vasculitis whose initial manifestation was axillary lymphadenopathy. The axillary lymph node needle biopsy specimens had reactive hyperplasia. One year later, the bilateral inguinal lymph nodes had similar morphological and structural changes, and laboratory test results showed renal insufficiency. A renal biopsy revealed the presence of sclerotic glomeruli, crescentic glomeruli, and fibrous crescentic glomeruli, but no deposition of immunocomplex or complement. Finally, the patient was treated with prednisone and mycophenolate mofetil. As the laboratory indicators normalized, so did the sizes of the axillary lymph nodes. A subsequent laboratory examination showed that in addition to urine protein all indicators had normalized, ultrasonography showed slight enlargement of unilateral axillary lymph nodes and normal hilum structure. Conclusions: Superficial lymphadenopathy is very rare in ANCA-associated systemic vasculitis. Studying this case improves our understanding of the initial manifestations of ANCA-associated vasculitis and may help provide accurate early diagnosis, thus allowing timely treatment and improved patient prognosis.
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Lhotta, Karl, Wolfgang Vogel, Thomas Meisl, Martina Buxbaum, Ulrich Neyer, Christoph Sandholzer, and Paul König. "α1-Antitrypsin Phenotypes in Patients with Anti-Neutrophil Cytoplasmic Antibody-Positive Vasculitis." Clinical Science 87, no. 6 (December 1, 1994): 693–95. http://dx.doi.org/10.1042/cs0870693.
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1. The genetic background of anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis remains largely unknown. Recently a very high prevalence of medium and severe deficiency of α1-antitrypsin was described in a small group of patients with Wegener's granulomatosis and c-ANCA. c-ANCAs are autoantibodies against proteinase 3, and α1-antitrypsin is the main inhibitor of this enzyme. 2. α1-Antitrypsin phenotypic polymorphism was determined by isoelectric focusing in 32 patients with c-ANCA-associated systemic vasculitis. Twenty-nine patients had Wegener's disease, two had microscopic polyarteritis and one suffered from idiopathic rapidly progressive glomerulonephritis. 3. Two patients were homozygous PiZZ and three were heterozygous PiMZ. These phenotype frequencies differed significantly from expected values, assuming Hardy-Weinberg equilibrium (P < 0.01). Compared with a control group of 868 healthy blood donors, these results meant a significant increase in the PiZ allele (0.0138 versus 0.1094, P< 0.001). 4. Furthermore, the serum of 47 patients with severe α1-antitrypsin deficiency (PiZZ) was tested for the presence of ANCA. All sera were negative for c-ANCA and p-ANCA. None of the patients showed clinical signs of systemic vasculitis. 5. In conclusion, these data indicate that α1-antitrypsin deficiency, despite being significantly more common in patients with c-ANCA-associated systemic vasculitis, is only a minor genetic risk factor for the development of this disease.
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Shimizu, Hayato, and Hiroaki Nishioka. "ANCA-associated vasculitis with muscle involvement mimicking polymyalgia rheumatica." BMJ Case Reports 17, no. 6 (June 2024): e257828. http://dx.doi.org/10.1136/bcr-2023-257828.
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We report a case of a woman in her early 80s with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis presented as myalgia mimicking polymyalgia rheumatica (PMR). She had positive results for the Neer and Hawkins-Kennedy impingement tests, and a normal serum creatine kinase (CK) concentration. At first, we suspected PMR; however, the patient did not strictly meet the classification criteria. Electromyography revealed an abnormal myogenic pattern, and muscle MRI revealed intramuscular and fascial hyperintensity. Moreover, chest CT revealed interstitial lung disease, and test results for ANCAs were positive. We diagnosed the patient with ANCA-associated vasculitis based on the criteria and treated her with corticosteroids and rituximab. Thus, ANCA-associated vasculitis can cause muscle involvement without elevation of the CK concentration and mimic PMR.
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Kluth, DC, and J. Hughes. "ANCA-associated systemic vasculitis (AASV)." Journal of the Royal College of Physicians of Edinburgh 37, no. 2 (June 2007): 128–34. https://doi.org/10.1177/1478271520073702015.
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Anti-neutrophil cytoplasmic antibody-associated systemic vasculitis is a multi-system disease that can present to a number of medical specialties, and where early identification and treatment improves outcome. The vasculitides are commonly divided into WG, MPA, and CSS. This review will revise the common clinical presentations of these diseases and initial investigations. Anti-neutrophil cytoplasmic antibodies have provided a valuable test to aid in diagnosis and there is increasing clinical and experimental evidence that suggests they have an important role in the pathogenesis of disease. Anti-neutrophil cytoplasmic antibodies are directed at either MPO or PR3 which are present in the granules and on the cell surface of neutrophils. They can be detected by indirect immunofluoresence and ELISA-based assay, and both are essential when vasculitis is suspected. The antibodies are able to activate neutrophils and promote injury, and recent data have shown they can directly cause glomerulonephritis and systemic vasculitis in animal models. Diagnosis of AASV is made using clinical features, ANCA test, and relevant tissue biopsy. Treatment with cytotoxics has transformed the prognosis but until recently there has been limited trial evidence to determine which regime is best. This article will review a range of studies published over the last five years and preliminary evidence from ongoing studies to give an outline of current practice for immunosuppression and monitoring of disease. There is also now a range of biological therapies available including anti-TNF and anti-B-cell antibodies, which may have an increasing role as either adjunctive therapy or new regimes to induce remission.
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Almaani, Salem, Lynn A. Fussner, Sergey Brodsky, Alexa S. Meara, and David Jayne. "ANCA-Associated Vasculitis: An Update." Journal of Clinical Medicine 10, no. 7 (April 1, 2021): 1446. http://dx.doi.org/10.3390/jcm10071446.
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Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) represents a group of small vessel vasculitides characterized by granulomatous and neutrophilic tissue inflammation, often associated with the production of antibodies that target neutrophil antigens. The two major antigens targeted by ANCAs are leukocyte proteinase 3 (PR3) and myeloperoxidase (MPO). AAV can be classified into 3 categories based on patterns of clinical involvement: namely, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic GPA (EGPA). Clinically, AAV involves many organ systems including the lungs, kidneys, skin, and nervous system. The prognosis of AAV has improved dramatically due to advances in the understanding of its pathogenesis and treatment modalities. This review will highlight some of the recent updates in our understanding of the pathogenesis, clinical manifestations, and treatment options in patients with AAV focusing on kidney involvement.
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Flossmann, Oliver, Annelies Berden, Kirsten de Groot, Chris Hagen, Lorraine Harper, Caroline Heijl, Peter Höglund, et al. "Long-term patient survival in ANCA-associated vasculitis." Annals of the Rheumatic Diseases 70, no. 3 (November 24, 2010): 488–94. http://dx.doi.org/10.1136/ard.2010.137778.
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BackgroundWegener's granulomatosis and microscopic polyangiitis are antineutrophil cytoplasm antibodies (ANCA)-associated vasculitides with significant morbidity and mortality. The long-term survival of patients with ANCA associated vasculitis treated with current regimens is uncertain.ObjectiveTo describe the long-term patient survival and possible prognostic factors at presentation in an international, multicentre, prospectively recruited representative patient cohort who were treated according to strictly defined protocols at presentation and included the full spectrum of ANCA-associated vasculitis disease.MethodsOutcome data were collected for 535 patients who had been recruited at the time of diagnosis to four randomised controlled trials between 1995 and 2002. Trial eligibility was defined by disease severity and extent, covered the spectrum of severity of ANCA-associated vasculitis and used consistent diagnostic criteria. Demographic, clinical and laboratory parameters at trial entry were tested as potential prognostic factors in multivariable models.ResultsThe median duration of follow-up was 5.2 years and 133 (25%) deaths were recorded. Compared with an age- and sex-matched general population there was a mortality ratio of 2.6 (95% CI 2.2 to 3.1). Main causes of death within the first year were infection (48%) and active vasculitis (19%). After the first year the major causes of death were cardiovascular disease (26%), malignancy (22%) and infection (20%). Multivariable analysis showed an estimated glomerular filtration rate <15 ml/min, advancing age, higher Birmingham Vasculitis Activity Score, lower haemoglobin and higher white cell count were significant negative prognostic factors for patient survival.ConclusionPatients with ANCA-associated vasculitis treated with conventional regimens are at increased risk of death compared with an age- and sex-matched population.
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Yap, Jin Yi, Mohtar Ibrahim, and Zunaina Embong. "An atypical presentation of ANCA-associated retinal vasculitis in a young Malay woman." Malaysian Journal of Ophthalmology 1, no. 3 (October 29, 2019): 214–21. http://dx.doi.org/10.35119/myjo.v1i3.60.
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We describe a case of antineutrophil cytoplasmic antibody (ANCA)-associated retinal vasculitis in a 21-year-old previously healthy Malay woman, who presented to us with complaints of sudden painless loss of vision in her left eye. Her vision upon presentation was counting fingers and her fundus examination showed retinal vasculitis and ischemic changes. Fundus fluorescein angiography showed leakage from the vasculitic retinal vessels, resulting in macular oedema. All investigations were normal, except that a full blood test showed eosinophilia and an autoimmune screening revealed positive perinuclear staining-ANCA, which led us to diagnose ANCA-associated retinal vasculitis. The patient was comanaged with the rheumatology team. She was started on a high-dose intravenous steroid followed by a tapering dose of an oral steroid. Improvement was noted from the resolution of macular oedema evident on optical coherence tomography of the macula. However, vision remained poor and unchanged.
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Karras, Alexandre, Hélène Lazareth, and Sophie Chauvet. "B-cell treatment in ANCA-associated vasculitis." Rheumatology 59, Supplement_3 (April 29, 2020): iii68—iii73. http://dx.doi.org/10.1093/rheumatology/kez605.
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Abstract The pivotal role of B-cells in ANCA-associated vasculitis has been suggested by experimental data that demonstrate the direct pathogenicity of ANCAs. Rituximab (RTX), an anti-CD20 monoclonal antibody that targets B-cells, has proven its efficacy for induction of remission in severe ANCA vasculitis. RTX is equivalent to CYC for induction of remission, and is probably superior in relapsing patients. Long-term B cell depletion by prolonged RTX treatment has been shown to significantly reduce the relapse rate, when compared with AZA maintenance therapy. Biomarkers, such as B-cell subpopulations or ANCA monitoring, may help the clinician to determine the optimal dose and duration of RTX therapy.
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Fatthi, Dalal Abdullah. "The effect of various forms of treatment of vasculitis on C3, C4 and C5a complement levels in infants and children attending Assiut University Children Hospital (AUCH)." E3S Web of Conferences 391 (2023): 01135. http://dx.doi.org/10.1051/e3sconf/202339101135.
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Vasculitis is an umbrella term for various and heterogeneous disorders sharing the presence of inflammation of blood vessel walls (Geetha & Jefferson, 2020). Immune cell infiltrates involve the presence of leukocytes in the vessel with immune-complex deposition, which implies the activation of the complement system and then the swelling and destruction of vessel mural structures. The lumen is narrowed or occluded leading to ischemia and necrosis (Chimenti et al., 2015). It produces local symptoms resulting in hypoperfusion, infarction and hemorrhage and systemic symptoms with an increase in acute phase reactants (Salvador, 2020). According to the size of the blood vessel affected and the distribution of vascular injury, pediatric vasculitis is classified into small vessel vasculitis as in Henochonlein Purpura (HSP), medium-sized vessel vasculitis as in Kawasaki disease and large vessel vasculitides affecting the aorta and its proximal branches. Some forms of small vessel vasculitis are characterized by the presence of antineutrophil cytoplasmic antibodies (ANCAs), whereas others are associated with immune complex deposition in affected tissues. Clinical presentation supported by specific laboratory test, imaging, and confirmatory histology are necessary in order to perform vasculitides’ diagnosis (Sivaraman et al., 2020). To assess the severity of vasculitis and response to treatment, urinary biomarkers such as albumin/creatinine ratio (A/C ratio) in urine have been proved to be easily done and correlated well with both clinical and serological results (Zhang, 2020). Aim of Work: The aim of this study is to assess the effect of various forms of drugs, used in treatment of vasculitis, on the serum levels of complements (C3, C4, and C5a) and Antineutrophil cytoplasmic antibodies (ANCA), in infants and children attending Assiut University Children Hospital (AUCH). Conclusion: In conclusion, cases on combined Methotrexate and Steroid therapy scored best regarding the lowering of C5a level in serum, however, in our cases as a whole, the level of C5a didn’t differ significantly from its level in control. Although the 3 arms of therapy used in this study had good effect on C3 & C4 levels, yet their effect on C5a level was not significant from control. This finding could be explained by the fact that most of our studied cases were of the immune-complex deposition diseases type (which have no effect on C5a) and were ANCA negative. Therefore, C5a inhibitor drugs probably will not be suitable in treatment of most common causes of pediatric vasculitis encountered in this series. Perhaps, this type of therapy might be used in ANCA-Positive associated vasculitides, e.g. Wegener’s Granulomatosis. Further research with bigger number of ANCA positive cases is needed to decide whether such cases could benefit from the use of C5a inhibitors or not.
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ALem, Leila. "Propylthiouracil-induced ANCA-positive vasculitis in Graves’ disease." Journal of Parathyroid Disease 11 (August 31, 2023): e11241. http://dx.doi.org/10.34172/jpd.2023.11241.
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Implication for health policy/practice/research/medical education: Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis is an infrequent autoimmune disease that involves small vessels. Vasculitis triggers include infections, drugs, and chemicals. Propylthiouracil (PTU) is the most reported drug implicated in the induction of ANCA-associated vasculitis. The involvement of hematological systems, skin, gastrointestinal systems, kidneys, musculoskeletal systems, lungs, and neurological systems are seen in PTU-induced vasculitis. The exact pathogenesis of ANCA induction and PTU-induced vasculitis is not fully understood. Diagnosing PTU-induced ANCA-positive vasculitis involves a combination of clinical, laboratory, imaging, and histopathological findings.
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Sun, Xiao-Jing, Zhi-Ying Li, and Min Chen. "Pathogenesis of anti-neutrophil cytoplasmic antibody-associated vasculitis." Rheumatology and Immunology Research 4, no. 1 (March 1, 2023): 11–21. http://dx.doi.org/10.2478/rir-2023-0003.
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Abstract Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) encompasses a group of potentially life-threatening disorders characterized by necrotizing small vessel vasculitis with positive serum ANCA. To date, the pathogenesis of AAV has not been fully elucidated, but remarkable progress has been achieved in the past few decades. In this review, we summarize the mechanism of AAV. The pathogenesis of AAV involves various factors. ANCA, neutrophils, and the complement system play key roles in disease initiation and progression, forming a feedback amplification loop leading to vasculitic injury. Neutrophils activated by ANCA undergo respiratory burst and degranulation, as well as releasing neutrophils extracellular traps (NETs), thus causing damage to vascular endothelial cells. Activated neutrophils could further activate the alternative complement pathway, leading to the generation of complement 5a (C5a), which amplifies the inflammatory response by priming neutrophils for ANCA-mediated overactivation. Neutrophils stimulated with C5a and ANCA could also activate the coagulation system, generate thrombin, and subsequently cause platelet activation. These events in turn augment complement alternative pathway activation. Moreover, disturbed B-cell and T-cell immune homeostasis is also involved in disease development. In-depth investigation in pathogenesis of AAV might help to offer more effective targeted therapies.
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Khan, Md Ali Akbar, Md Titu Miah, Sharar Naiarin Haque, Shah Mubdi Un Naafi, and Sakib Abrar. "A Case of C-ANCA Associated Vasculitis Presenting With Localised Skin Thickening." Journal of Dhaka Medical College 31, no. 1 (May 3, 2023): 158–62. http://dx.doi.org/10.3329/jdmc.v31i1.65490.
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ANCA vasculitis, caused by antibodies named ANCA (anti-neutrophilic cytoplasmic autoantibodies), is an autoimmune disorder affecting small blood vessels of the body. Among p-ANCA and c- ANCA, c-ANCA (cytoplasmic ANCA) usually targets proteinase three inside neutrophils, causing inflammation of blood vessels. Herein, we report a case of a 56-year-old female patient with c- ANCA-associated vasculitis. The patient presented with a localized skin thickening on the medial aspect of the left thigh and an oral ulcer. Skin biopsy shows erythema induratum (nodular vasculitis). After several investigations, we found c-ANCA positive and concluded the diagnosis as c-ANCA-associated vasculitis. We treat the patient with injectable methylprednisolone, injectable cyclophosphamide, and Mesna. Then we gave oral mycophenolate mofetil and prednisolone. J Dhaka Med Coll. 2022; 31(1) : 158-162
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Hakroush, Samy, Ingmar Alexander Kluge, Eva Baier, Désirée Tampe, and Björn Tampe. "Relevance of Complement C4 Deposits Localized to Distinct Vascular Compartments in ANCA-Associated Renal Vasculitis." International Journal of Molecular Sciences 23, no. 22 (November 18, 2022): 14325. http://dx.doi.org/10.3390/ijms232214325.
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Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a small-vessel vasculitis affecting multiple organ systems, including the kidney. Small vessels in the kidney include small-sized arteries, capillaries, and venules. Intrarenal C4 deposits are now increasingly recognized as a potential marker and pathogenic mechanism of autoantibody-mediated tissue damage in ANCA-associated renal vasculitis. We here describe the relevance of complement C4 deposits localized to distinct vascular compartments in a cohort of biopsy-proven ANCA-associated renal vasculitis. A cohort of 43 biopsy-proven cases of ANCA-associated renal vasculitis with myeloperoxidase (MPO) or proteinase 3 (PR3) seropositivity were retrospectively enrolled in a single-center observational study. Univariate and multivariate regression analysis was performed to identify parameters associated with intrarenal C4 deposits in ANCA-associated renal vasculitis. We here show that C4 deposits localize to distinct vascular compartments in ANCA-associated renal vasculitis, and provide evidence for an association with better short-term survival (p = 0.008), implicating that this subgroup had a superior response to remission induction therapy. Second, C4 deposits in interlobular arteries were associated with eosinophilic infiltrates in renal vasculitis with MPO-ANCA seropositivity (p = 0.021). In renal vasculitis positive for MPO-ANCA, the absence of C4 deposits in the glomerular tuft was associated with sclerotic class ANCA-associated renal vasculitis (p < 0.001), and tubular RBC casts (p = 0.024). Fourth, complement C4 in interlobular arteries is associated with tubular atrophy specifically in renal vasculitis with PR3-ANCA seropositivity (p = 0.006). Finally, complement C4 deposits in peritubular capillaries associated specifically with hyaline casts in cases positive for PR3-ANCA (p = 0.025), implicating a role in tubular injury. Interestingly, C4 deposits were localized to distinct vascular compartments independent of the systemic activation of the complement system, reflected by the consumption of respective serum complement molecules in ANCA-associated renal vasculitis. In summary, we here show that C4 deposits localize to distinct vascular compartments in ANCA-associated renal vasculitis, and provide evidence for an association with survival and distinct histopathological lesions. Considering recent advances in AAV therapy with the emergence of new therapeutics that inhibit complement activation, we here provide novel insights into complement C4 as a potential marker to identify patients who may benefit most from these drugs. Thus, our results may contribute to a more personalized treatment approach of AAV depending on the relevance of distinct intrarenal complement deposits.
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Burlutskaya, A. V., N. V. Savelyeva, and N. S. Тaran. "ANCA-associated vasculitis in a 14 years-old patient: a clinical case." Kuban Scientific Medical Bulletin 27, no. 5 (October 14, 2020): 184–94. http://dx.doi.org/10.25207/1608-6228-2020-27-5-184-194.
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Background. ANCA-associated systemic vasculitis is a rare childhood disease. Antineutrophil cytoplasmic autoantibodies (ANCA)-related vasculitises include microscopic polyangiitis, granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis. Their rarity often leads to a late diagnosis, rapid disability and high mortality in patients due to aggressive respiratory, pulmonary lesion and renal failure.Clinical Case Description. The patient suffered from a recurrent bronchoobstructive syndrome with signs of respiratory failure, obscure origin fever and chronic rhinitis with nasal bleeding for 6 months. The patient was diagnosed with obstructive bronchitis (putative bronchial asthma debut), received antibacterial therapy and inhalation bronchodilators without stable improvement during the entire period. Skin haemorrhages and arthralgia stimulated diagnostic research to establish ANCA-associated systemic vasculitis (presence of proteinase 3-specifi c ANCAs in titre 1/80). CT lung scanning revealed frosted glass foci of reduced pulmonary pneumatisation and signs of bilateral bronchoobstruction. Immunosuppressive therapy with glucocorticosteroids (methylprednisolone pulse therapy No. 3, 1000 mg intravenously on alternate days, subsequent per os administration of 1 mg/kg/day) and cyclophosphamide (500 mg intravenously once per 28 days) was prescribed. This led to the positive dynamics with eliminated fever and skin haemorrhages, as well as essentially reduced signs of respiratory failure.Conclusion. Diagnosis of systemic vasculitis is often complicated and long-term due to commonly non-specifi c debut symptoms of autoimmune disorders. In the described case, the fi rst 6 months of illness displayed intoxication and bronchoobstruction with signs of respiratory failure. Haemorrhagic rashes, arthralgias and the presence of ANCAs are proxy to vasculitis. Standard immunosuppressive therapy for ANCA-associated vasculitis improved the patient’s condition.
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Folci, Marco, Giacomo Ramponi, Dana Shiffer, Aurora Zumbo, Michele Agosti, and Enrico Brunetta. "ANCA-Associated Vasculitides and Hematologic Malignancies: Lessons from the Past and Future Perspectives." Journal of Immunology Research 2019 (May 6, 2019): 1–9. http://dx.doi.org/10.1155/2019/1732175.
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The purpose of this paper is to collect and summarize all evidences relating to an association between ANCA-associated vasculitides (AAVs) and hematologic malignancies, in the form of either a paraneoplastic vasculitis or leukemias and lymphomas developing on a preexisting vasculitis. Additionally, the role of cyclophosphamide in vasculitis treatment has been assessed and compared to rituximab. Paraneoplastic AAV seems to be an uncommon presentation of hemopathies. Hematologic malignancy risk in AAV is more likely to be increased by cyclophosphamide, although not yet definitely proven. Furthermore, the pathogenesis of ANCA-associated vasculitis has been reviewed with particular emphasis on the role of proteinase 3 (PR3) in fuelling granulomatosis with polyangiitis (GPA) inflammation. PR3 is a bactericidal protein expressed by neutrophilic granules and on their plasma membrane. Derangements in its expression and function have been linked to leukemias and GPA alike. PR3-derived PR1 peptide is being studied as an immunotherapy target in leukemia and multiple myeloma. This study is aimed at bringing together various evidences from the field of immunological and hematological research, at exposing contradictions, and at revealing novel insights on the association between ANCA-associated vasculitis and hematologic malignancies.
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Thongprayoon, Charat, Wisit Kaewput, Boonphiphop Boonpheng, Patompong Ungprasert, Tarun Bathini, Narat Srivali, Saraschandra Vallabhajosyula, et al. "Impact of ANCA-Associated Vasculitis on Outcomes of Hospitalizations for Goodpasture’s Syndrome in the United States: Nationwide Inpatient Sample 2003–2014." Medicina 56, no. 3 (March 1, 2020): 103. http://dx.doi.org/10.3390/medicina56030103.
Full textAbstract:
Background and objectives: Goodpasture’s syndrome (GS) is a rare, life-threatening autoimmune disease. Although the coexistence of anti-neutrophil cytoplasmic antibody (ANCA) with Goodpasture’s syndrome has been recognized, the impacts of ANCA vasculitis on mortality and resource utilization among patients with GS are unclear. Materials and Methods: We used the National Inpatient Sample to identify hospitalized patients with a principal diagnosis of GS from 2003 to 2014 in the database. The predictor of interest was the presence of ANCA-associated vasculitis. We tested the differences concerning in-hospital treatment and outcomes between GS patients with and without ANCA-associated vasculitis using logistic regression analysis with adjustment for other clinical characteristics. Results: A total of 964 patients were primarily admitted to hospital for GS. Of these, 84 (8.7%) had a concurrent diagnosis of ANCA-associated vasculitis. Hemoptysis was more prevalent in GS patients with ANCA-associated vasculitis. During hospitalization, GS patients with ANCA-associated required non-significantly more mechanical ventilation and non-invasive ventilation support, but non-significantly less renal replacement therapy and plasmapheresis than those with GS alone. There was no significant difference in in-hospital outcomes, including organ failure and mortality, between GS patients with and without ANCA-associated vasculitis. Conclusions: Our study demonstrated no significant differences between resource utilization and in-hospital mortality among hospitalized patients with coexistence of ANCA vasculitis and GS, compared to those with GS alone.
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Takeuchi, Sora, Tamihiro Kawakami, Tatsuro Okano, Haruki Shida, Daigo Nakazawa, Utano Tomaru, Akihiro Ishizu, and Takafumi Kadono. "Elevated Myeloperoxidase-DNA Complex Levels in Sera of Patients with IgA Vasculitis." Pathobiology 89, no. 1 (November 23, 2021): 23–28. http://dx.doi.org/10.1159/000519869.
Full textAbstract:
Introduction: IgA vasculitis is a systemic disease that results from the entrapment of circulating IgA-containing immune complexes in small-vessel walls in the skin, kidneys, and gastrointestinal tract. An excessive formation of neutrophil extracellular traps (NETs) is involved in the pathogenesis of vasculitis, especially in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. This study aimed to clarify whether NETs are implicated in IgA vasculitis. Methods: Twenty-two patients with IgA vasculitis and 4 healthy volunteers were enrolled in this study. Serum levels of myeloperoxidase (MPO)-DNA complex, a fragment derived from NETs, were determined by enzyme-linked immunosorbent assay (ELISA), and the association between MPO-DNA complex levels and clinical parameters was examined. The presence of the ANCA was also assessed by ELISA specific for MPO and proteinase 3 (PR3) and indirect immunofluorescence (IIF), followed by assessing the differences in clinical parameters with and without the ANCA. Results: Serum MPO-DNA complex levels were significantly higher in patients with IgA vasculitis than those in healthy controls. A significant positive correlation between the serum MPO-DNA complex and IgA levels was noted. Interestingly, 63.6% of IgA vasculitis patients were ANCA-positive in IIF with an atypical pattern, whereas neither MPO-ANCA nor PR3-ANCA was detected by ELISA. These findings indicated that some IgA vasculitis patients possessed the so called minor ANCA. Serum IgA and MPO-DNA complex levels and the frequency of hematuria in the minor ANCA-positive group were significantly higher than in the minor ANCA-negative group. Conclusion: The collective findings suggested that NETs are certainly involved in the pathogenesis of IgA vasculitis.