Clinical features and outcomes of patients with microscopic polyangiitis: experience of two centers
1Okmeydani Trainning and Research Hospital, Chest Diseases Clinic Kaptan Pasa Mahallesi, Istanbul, Turkey
2Izmir Metropolitan Municipality Hospital, Chest Diseases Clinic Gaziler Caddesi, Izmir, Turkey
DOI: 10.22514/sv.2020.16.0100 Vol.17,Issue 2,March 2021 pp.168-173
Published: 08 March 2021
Background and Aim: Microscopic polyangiitis (MPA) is a rare necrotizing vasculitis that involves predominantly the small-caliber blood vessels. In this retrospective study, we aimed to evaluate the clinical findings, prognostic factors, and outcomes of MPA patients followed in two different centers.
Material and Method: In this retrospective study, a total of 38 patients diagnosed and followed with MPA in two different centers between January 2005 and December 2019 were included. Medical records of the patients were reviewed retrospectively. The diagnoses were based on the definition of MPA in the Chapel Hill consensus conference (CHCC-2012). In addition to the socio-demographic characteristics, clinical features, laboratory and immunological parameters; time passed for the diagnosis, treatment, outcomes, and follow-up time were recorded for each patient. The Five Factor Scores (FFS; Revised FFS 2009) and Birmingham Vasculitis Activity Score (BVAS; Version 3) were applied in all patients.
Results: In a total of 38 patients (10 females, 28 male) were included in the study. The mean age of the participants was 54.52 ± 8.21 (range: 32-74) years. The mean time passed for the diagnosis was 2.93 ± 2.03 (0.50-11) months and the mean follow-up period was 55.0 ± 30.51 (2-124) months. At admission, the mean BVAS was 18.28 ± 5.73 (8-35). The FFS results were as follows; 0 in 4 (10.5%) patients, 1 in 27 (71.1%) patients, 2 in 7 (18.4%) patients. The most common symptoms at admission were dyspnea, cough, and hemoptysis. Renal failure was present in 31 (81.5%) of the patients, at admission. The most common immunologic finding was the p-ANCA positivity in 26 (68.4%) patients. Among all patients, 25 (65.8 %) did not have any other attacks after the diagnosis. In follow-ups, 12 (31.5%) patients were passed away and vasculitis associated mortality rate was 18.4%. The effects of some factors on mortality including age ≥ 65 years, male gender, renal involvement, p-ANCA positivity, decreased oxygen saturation (< 90%), having anemia at admission, having FEV1/FVC < 70 in spirometry, having a FFS score of 2, and having a BVAS > 18 were examined (Table 6). Only having an FFS score of 2 and BVAS > 18 were determined as the factors significantly affecting mortality (P: 0.01).
Conclusion: Pulmonary symptoms are more predominant in MPA. Though many patients did not have any other attacks after the diagnosis, the vasculitis associated mortality may reach about 20%. BVAS and FFS are still the best determinants of mortality.
Microscopic polyangiitis; Autoimmune vasculitis; Birmingham Vasculitis Activity Score (BVAS); Five Factor Scores (FFS)
Dasdemir Ilkhan Gulay,Celikhisar Hakan. Clinical features and outcomes of patients with microscopic polyangiitis: experience of two centers. Signa Vitae. 2021. 17(2);168-173.
 Berti A, Cornec D, Crowson CS, Specks U, Matteson EL. The epidemiology of antineutrophil cytoplasmic autoantibody-associated vasculitis in olmsted county, minnesota: A twenty-year US population-based study. Arthritis & Rheumatology. 2017; 69: 2338-2350.
 Greco A, De Virgilio A, Rizzo MI, Gallo A, Magliulo G, Fusconi M, et al. Microscopic polyangiitis: Advances in diagnostic and therapeutic approaches. Autoimmunity Reviews. 2015; 14: 837-844.
 Karras A. Microscopic polyangiitis: New insights into pathogenesis, clinical features and therapy. Seminars in Respiratory and Critical Care Medicine. 2018; 39: 459-464.
 Weiner M, Segelmark M. The clinical presentation and therapy of diseases related to anti-neutrophil cytoplasmic antibodies (ANCA). Autoimmunity Reviews. 2016; 15: 978-982.
 Jeannette, JC. Overview of the 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides; Clinical and Experimental Nephrology, 2013; 17: 603-606.
 Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P, et al. The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore). 2011; 90: 19-27.
 Mukhtyar C, Lee R, Brown D, Carruthers D, Dasgupta B, Dubey S, et al. Modification and validation of the Birmingham Vasculitis Activity Score (version 3). Annals of the Rheumatic Diseases. 2009; 68: 1827-1832.
 Chung SA, Seo P. Microscopic polyangiitis. Rheumatic Disease Clinics of North America. 2010; 36: 545-558.
 Savage CO, Winearls CG, Evans DJ, Rees AJ, Lockwood CM. Micro-scopic polyarteritis: presentation, pathology and prognosis. Quarterly Journal of Medicine. 1985; 56: 467-483.
 Franks TJ, Koss MN. Pulmonary capillaritis. Current Opinion in Pulmonary Medicine. 2000; 6: 430-435.
 Kluger N, Pagnoux C, Guillevin L, Francès C, French Vasculitis Study Group. Comparison of cutaneous manifestations in systemic polyarteritis nodosa and microscopic polyangiitis. British Journal of Dermatology. 2008; 159: 615-620.
 Ueda S, Matsumoto M, Ahn T, Adachi S, Oku K, Takagi M, et al. Microscopic polyangiitis complicated with massive intestinal bleeding. Journal of Gastroenterology. 2001; 36: 264-270.
 Zhang W, Zhou G, Shi Q, Zhang X, Zeng XF, Zhang FC. Clinical analysis of nervous system involvement in ANCA-associated systemic vasculitides. Clinical and Experimental Rheumatology. 2009; 27: S65-S69.
 Harigai M, Tsutsumino M, Takada H, Nagasaka K. Molecular targeted therapies for microscopic polyangiitis and granulomatosis with polyangi-itis. Korean Journal of Internal Medicine. 2019; 34: 492-503.
 Yates M, Watts R. ANCA-associated vasculitis. Clinical Medicine (London). 2017; 17: 60-64.
 Abe Y, Tamura N, Yang KS, Matsuoka J, Kon T, Yamaji K, et al. Predictive factors for mortality in elderly Japanese patients with severe microscopic polyangiitis: A retrospective single-center study. Modern Rheumatology. 2017; 27: 315-319.
 Park HJ, Park JY, Jung SM, Song JJ, Park YB, Lee SW. Fibrosis-4 index at diagnosis is associated with all-cause mortality in patients with microscopic polyangiitis and granulomatosis with polyangiitis. BMC Gastroenterology. 2019; 19: 90.
 Solans-Laqué R, Rodriguez-Carballeira M, Rios-Blanco JJ, Fraile G, Sáez-Comet L, Martinez-Zapico A, et al. Comparison of the Birmingham vasculitis activity score and the five-factor score to assess survival in antineutrophil cytoplasmic antibody-associated vasculitis: a study of 550 patients from spain (REVAS Registry). Arthritis Care & Research (Hoboken). 2020; 72: 1001-1010.
 Gayraud M, Guillevin L, le Toumelin P, Cohen P, Lhote F, Casassus P, et al. Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: analysis of four prospective trials including 278 patients. Arthritis & Rheumatology. 2001; 44: 666-675.
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