Toevaluateclinicalvariantsandoutcomesofmicroscopic polyangiitis (MPA).
Material and methods
We recruited all consecutive patientswithMPA that was established according to the EMA algorythm and CHCC nomenclature (2012).
One hundred patients with MPA with a median follow-up of 28.1 months (1;231) were enrolled in our retrospective study. Kidney and lung disease were the most common manifestations of MPA (93.1% and 78.4%, respectively). Diffuse alveolar hemorrhage was reported in 30.4% of patients. In 53% of cases, kidney involvement was characterized by development of rapidly progressing glomerulonephritis (RPGN). One-year and 5-year survival was 95.9% and 91.5%, respectively. Riskofdeathwas significantly increased in patients with diffuse alveolar hemorrhage (odds ratio [OR] 14.3; 95% confidence interval [CI] 1,7-119,0, р=0.014). Diffusealveolarhemorrhagewasalsoassociated with a higher incidence of RPGN (OR 2.54, 95% CI 1.09-6.16), infections (OR 3.37, 95% CI 1.34-8.53), while BPGN was associated withahigherriskofend-stage renal disease (OR 1.85, 95% CI 1.40-28.84; р=0.002) that developed in 14 (13.7%) patients.
Long-term survival was high in our patients with MPA. Diffuse alveolar hemorrhage was the most significant predictor of death, while BPGN predicted renal survival.
Microscopic polyangiitis, diffuse alveolar hemorrhage, rapidly progressing glomerulonephritis, pulmonary fibrosis.