Vasculitis-Related Respiratory Disorders
Paul C. Stillwell, MD, FAAP, and Robin R. Deterding, MD
•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.
|Small Vessels||Medium Vessels||Large Vessels|
|(Arterioles and Capillaries)||(Arteries and Arterioles)||(Aorta and Major Branches)|
|GPA||Polyarteritis nodosa||Giant cell arteritis|
|EGPA||Kawasaki disease||Takayasu arteritis|
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.