Abstract
Many vasculitis syndromes can cause respiratory disease manifestations. Large and medium size vessel vasculitides are briefly reviewed. The small vessel vasculitides most frequently affect the respiratory tract. They are divided into those associated with detectable tissue immune deposits versus those without (pauci-immune). Pauci-immune small vessel vasculitides usually have circulating anti-neutrophil cytoplasmic autoantibodies (ANCA) and are consequently called ANCA-associated vasculitides. The spectrum of their respiratory manifestations and their management is described in detail. Diffuse alveolar hemorrhage (DAH) from disruption of alveolar-capillary basement membranes is caused by immune-mediated disease processes with or without prominent capillaritis, by direct toxic effects of inhaled or blood-borne agents, by coagulopathies or by capillary stress failure. The ANCA-associated vasculitides causing capillaritis are the most common immune-mediated cause of DAH, but many other systemic autoimmune disorders can also rarely cause capillaritis. A systematic diagnostic and management approach to the patient with DAH is described.
Original language | English (US) |
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Title of host publication | Encyclopedia of Respiratory Medicine, Second Edition |
Publisher | Elsevier |
Pages | 341-357 |
Number of pages | 17 |
Volume | 3 |
ISBN (Electronic) | 9780081027240 |
ISBN (Print) | 9780081027233 |
DOIs | |
State | Published - Jan 1 2021 |
Keywords
- ANCA
- Alveolar hemorrhage
- Anti-GBM disease
- Antineutrophil cytoplasmic antibodies
- Capillaritis
- Eosinophilic granulomatosis with polyangiitis
- Granulomatosis with polyangiitis
- Immunosuppression
- Microscopic polyangiitis
- Plasma exchange
- Vasculitis
ASJC Scopus subject areas
- General Medicine