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Microscopic polyangiitis (MPA) is one of several vasculitides that affects the lung. Its most common pulmonary complication is diffuse alveolar haemorrhage, which carries a significant morbid risk. MPA association with pulmonary fibrosis (PF) has been increasingly reported. Do MPA patients with PF have different characteristics and evolution to those of MPA patients without PF?
In the retrospective review of 28 patients, high prevalence of pulmonary fibrosis in MPA patients (32%) was observed and most of MPA-PF patients had a poor outcome as compared to non-PF group. PF was often the first manifestation of the disease.