Bonus Material-06_Vasculitis_and_DAH_CHEST_Cartin-Ceba_2025

Pdf Summary

This review focuses on vasculitis and diffuse alveolar hemorrhage (DAH), particularly their pulmonary involvement, diagnosis, and management, aiming to deepen understanding of respiratory complications in systemic vasculitis.<br /><br />A clinical case illustrates a 68-year-old man presenting with hypoxemia, hemoptysis, anemia, renal impairment, and positive p-ANCA/MPO serology, consistent with microscopic polyangiitis (MPA), a type of ANCA-associated vasculitis (AAV). The differential diagnosis includes Goodpasture's disease, systemic lupus erythematosus, idiopathic pulmonary capillaritis, and others. Bronchoscopy findings showed bloody lavage with hemosiderin-laden macrophages indicating DAH.<br /><br />Vasculitis classification distinguishes large and medium vessel vasculitis (e.g., giant cell arteritis, Takayasu) from small vessel vasculitis, including ANCA-associated vasculitis (MPA, granulomatosis with polyangiitis [GPA], eosinophilic granulomatosis with polyangiitis [EGPA]). ANCA testing is helpful but must be interpreted carefully; p-ANCA/MPO and c-ANCA/PR3 antibodies aid diagnosis. New classification criteria from 2022 refine diagnosis of GPA, MPA, and EGPA based on clinical, lab, imaging, and biopsy data.<br /><br />DAH is common (25-27%) in severe AAV, often with rapidly progressive glomerulonephritis. Therapy includes immunosuppression with glucocorticoids, cyclophosphamide or rituximab, and potentially plasma exchange (though recent guidelines recommend against routine plasma exchange except in select cases). Avacopan, a C5a receptor inhibitor, shows promise in relapse prevention.<br /><br />EGPA differs by involving asthma, eosinophilia, and less frequent ANCA positivity; mepolizumab can be a steroid-sparing agent.<br /><br />Diffuse alveolar hemorrhage can result from immune-mediated capillaritis or non-immune mechanisms like capillary stress failure, such as in mitral stenosis patients presenting with DAH without systemic vasculitis.<br /><br />Overall, management depends on thorough clinical evaluation, serologic testing, and tailored immunosuppressive therapy, emphasizing a multidisciplinary approach to diagnosis and treatment in vasculitis-associated pulmonary disease.

Meta Tag

Concept Diffuse Alveolar Hemorrhage

Concept ANCA-Associated Vasculitis

Concept ANCA Test

Concept Microscopic Polyangiitis

Concept Granulomatosis with Polyangiitis

Keywords

vasculitis

diffuse alveolar hemorrhage

ANCA-associated vasculitis

microscopic polyangiitis

pulmonary involvement

hemoptysis

p-ANCA MPO

bronchoscopy

immunosuppression

glucocorticoids

Diffuse Alveolar Hemorrhage

ANCA-Associated Vasculitis

ANCA Test

Microscopic Polyangiitis

Granulomatosis with Polyangiitis