Bonus Material-06_Vasculitis_and_DAH_CHEST_Cartin-Ceba_2025

Pdf Summary

This board review presentation discusses respiratory complications of systemic vasculitis, focusing on ANCA-associated vasculitis (AAV) and diffuse alveolar hemorrhage (DAH). A case of a 68-year-old man with worsening dyspnea, hypoxemia, hemoptysis, anemia, renal impairment, and positive p-ANCA/MPO serology illustrates microscopic polyangiitis (MPA) as the likely diagnosis. Key vasculitis types are detailed including large and medium vessel (e.g., giant cell arteritis, Takayasu arteritis), and small vessel vasculitis subdivided into ANCA-associated (MPA, granulomatosis with polyangiitis GPA, eosinophilic granulomatosis with polyangiitis EGPA) and immune-complex mediated vasculitis (e.g., anti-GBM disease).<br /><br />The presentation highlights that ANCA testing is most specific when combined with clinical suspicion and that ANCA levels do not reliably track disease activity or predict relapse. Recent 2022 ACR/EULAR classification criteria for GPA and MPA include clinical features (nasal involvement, hearing loss, pulmonary nodules), serologies (c-ANCA/PR3, p-ANCA/MPO), and biopsy findings.<br /><br />DAH occurs in about a quarter of AAV cases, often in relapsing or PR3-positive patients, associated with worse prognosis. Plasma exchange (PLEX) does not routinely improve outcomes in severe AAV with glomerulonephritis or alveolar hemorrhage but may be considered in double-positive ANCA/anti-GBM cases or as salvage therapy. The C5a receptor inhibitor avacopan shows promise in reducing relapses.<br /><br />EGPA differs from GPA/MPA, with the vasculitis usually following asthma and eosinophilia, lower ANCA positivity (~30%), rare DAH, and steroid-sparing therapy with mepolizumab recommended in some cases.<br /><br />Diffuse alveolar hemorrhage can be immune-mediated with or without capillaritis or due to non-immune causes like capillary stress failure as seen in mitral stenosis-induced pulmonary hypertension. Diagnosis involves clinical presentation, imaging, bronchoalveolar lavage, and extensive serologic workup. Treatment centers on supportive care, immunosuppression (high-dose steroids, cyclophosphamide or rituximab), and occasionally adjunctive therapies.<br /><br />Overall, this review underscores the clinical and serologic heterogeneity of systemic vasculitis affecting the lungs, emphasizing tailored diagnosis and evolving therapeutic strategies.

Meta Tag

Concept ANCA-Associated Vasculitis

Concept Diffuse Alveolar Hemorrhage

Concept Granulomatosis with Polyangiitis

Concept Microscopic Polyangiitis

Concept Vasculitis

Keywords

systemic vasculitis

ANCA-associated vasculitis

diffuse alveolar hemorrhage

microscopic polyangiitis

granulomatosis with polyangiitis

eosinophilic granulomatosis with polyangiitis

p-ANCA

MPO serology

plasma exchange

avacopan

ANCA-Associated Vasculitis

Diffuse Alveolar Hemorrhage

Granulomatosis with Polyangiitis

Microscopic Polyangiitis

Vasculitis