Interstitial Lung Disease and Diffuse Alveolar Hemorrhage, the Two Key Pulmonary Manifestations in Microscopic Polyangiitis.

ABSTRACT: Microscopic polyangiitis (MPA) is an antineutrophil cytoplasmic antibody (ANCA)‒associated necrotizing vasculitis, which mainly affects small vessels in various organs, especially the lungs. The two key pulmonary manifestations, interstitial lung disease (ILD) and diffuse alveolar hemorrhage (DAH), increase the morbidity and death rate of patients with MPA. ILD is more common in MPA than in other ANCA-associated vasculitis subsets and is primarily associated with myeloperoxidase-ANCA. Unlike alveolar hemorrhage due to pulmonary capillaritis, ILD can initially manifest as isolated pulmonary fibrosis. Of note, its most frequent radiographic pattern is the usual interstitial pneumonia pattern, similar to the characteristic pattern seen in idiopathic pulmonary fibrosis. In this review we prese

SUBMITTER: Kim MJ 

PROVIDER: S-EPMC8497773 | biostudies-literature | 2021 Oct

REPOSITORIES: biostudies-literature