Vasculitis-Related Respiratory Disorders
Paul C. Stillwell, MD, FAAP, and Robin R. Deterding, MD
Introduction/Etiology/Epidemiology
•Pulmonary vasculitides include a variety of systemic diseases with vessel involvement in many organ systems, including the lung (Table 90-1).
•These can be classified according to the size of the vessels primarily involved or by the association with antineutrophil cytoplasmic antibodies (ANCA) (cytoplasmic ANCA [c-ANCA] and perinuclear ANCA [p-ANCA]) (Box 90-1).
•Some of the more common causes of vasculitis in childhood, such as Henoch-Schönlein purpura and Kawasaki disease, do not often involve the pulmonary vessels.
•The most common pulmonary-renal syndromes are compared in Table 90-2.
EGPA, eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome); GPA, granulomatous polyangiitis (formerly Wegener granulomatosis); HSP, Henoch-Schönlein purpura; MPA, microscopic polyangiitis.
Box 90-1. Vasculitides Classified According to the Size of Vessels Commonly Involved
| Small Vessels | Medium Vessels | Large Vessels |
| (Arterioles and Capillaries) | (Arteries and Arterioles) | (Aorta and Major Branches) |
| MPA | ||
| GPA | Polyarteritis nodosa | Giant cell arteritis |
| EGPA | Kawasaki disease | Takayasu arteritis |
| Goodpasture syndrome |
EGPA, eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome); GPA, granulomatous polyangiitis (formerly Wegener granulomatosis); MPA, microscopic polyangiitis. c-ANCA–positive vasculitis is most commonly GPA, whereas MPA and EGPA are more commonly p-ANCA positive.
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