Clinical features and outcomes of patients with microscopic polyangiitis: experience of two centers

2020 Signa Vitae  
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
more » ... tients 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.
doi:10.22514/sv.2020.16.0100 fatcat:i74piaizn5gvnprw56tz7c5yai