Vasculitis-Related Respiratory Disorders

Chapter 90


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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.


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EGPA, eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome); GPA, granulomatous polyangiitis (formerly Wegener granulomatosis); HSP, Henoch-Schönlein purpura; MPA, microscopic polyangiitis.


Aug 22, 2019 | Posted by in PEDIATRICS | Comments Off on Vasculitis-Related Respiratory Disorders
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