Poumon et vascularites

Respiratory symptoms and specific radiological features are observed in many vasculitides. They are associated with extra-thoracic signs (nasal and sinus obstruction, skin disease, and acute renal failure). The main vasculitides not associated with ANCA include Behçet's disease with pseudo-aneurysm and pulmonary artery thrombosis and Takayasu disease involving large vessels with thickening of the wall of the aorta and the pulmonary artery. The hypocomplementemic McDuffie syndrome is rare and includes extensive urticaria, obstructive respiratory disease, and finally Goodpasture's syndrome with acute glomerulonephritis and intra-alveolar hemorrhage. The three ANCA-associated vasculitides are microscopic polyangiitis (MPA) with glomerulonephritis and intra-alveolar hemorrhage, granulomatosis with polyangitis (GPA) and pauci-immune glomerulonephritis, and eosinophilic granulomatosis with polyangeitis (EGPA) characterised by athma, hypereosinophilia, and systemic manifestations. Treatment of ANCA-associated vasculitides is based on high-dose corticosteroids, immunosuppressive therapy, and occasionally rituximab.