ReviewTuberculosis and respiratory diseases2021
Interstitial Lung Disease and Diffuse Alveolar Hemorrhage, the Two Key Pulmonary Manifestations in Microscopic Polyangiitis.
Review in Tuberculosis and respiratory diseases, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 8 papers.
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Who cites it
8 citing papers in PubMed, 9 citations in OpenAlex.
- Article
- Vascular etiologies of unilateral pulmonary fibrosis: Case series and literature overview.Radiology case reports · 2026Article
- Recurrent Pulmonary Nodules, from Pulmonary Cryptococcosis to ANCAAssociated Vasculitis: A Case ReportCurrent medical imaging · 2026Article
- Article
- A comparative analysis of clinical phenotypes and outcomes in childhood interstitial lung disease due to surfactant dysfunction disorders: focusing on mutations in SFTPC, ABCA3, and NKX2-1 genes.Italian journal of pediatrics · 2025Article
- Clinical features, radiological findings and prognosis of microscopic polyangiitis with interstitial lung disease: a retrospective matched control study.BMC pulmonary medicine · 2024Article
- A versatile role for lung ultrasound in systemic autoimmune rheumatic diseases related pulmonary involvement: a narrative review.Arthritis research & therapy · 2024Review
- Lung Involvement in Pulmonary Vasculitis: A Radiological Review.Diagnostics (Basel, Switzerland) · 2024Review
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
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 present the pathogenesis, clinical manifestations, and radiographic and histopathologic features of ILD and DAH in MPA. We also briefly summarize the outcome and therapeutic options for the two conditions.
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