Paradigm Shift in the Management of ANCA-Associated Vasculitis: Pathogenetic Concepts Lead to Targeted Therapy
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چکیده
• Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome The respiratory system is commonly involved in all three syndromes, which share certain histopathologic and clinical features. Other features are unique and diseasedefining (Table 1). Diffuse alveolar hemorrhage (Figure 1, A1, see page 2) is a potentially life-threatening complication of each of these syndromes. Patients with diffuse alveolar hemorrhage often require ICU care. Caused by pulmonary capillaritis, diffuse alveolar hemorrhage affects 20 to 30 percent of patients with MPA and GPA, but less than 5 percent of patients with EGPA. Other clinical manifestations caused by necrotizing small vessel vasculitis and capillaritis, such as glomerulonephritis, palpable purpura, scleritis, or sensory and motor mononeuropathies, also can occur in all three syndromes. Granulomatous inflammation, which predominantly affects the respiratory tract, causes characteristic clinical features setting GPA and EGPA apart from MPA. The granulomatous inflammation is necrotizing and neutrophilic in GPA, but eosinophilic in EGPA. The necrotizing granulomatous inflammation of GPA can cause pulmonary nodules or mass lesions (Figure 1, B1, see page 2) and affect the large airways leading to subglottic and endobronchial stenoses (Figure 2, see page 3). Asthma and peripheral blood eosinophilia, the defining characteristics of EGPA, are not features of GPA or MPA. The type of ANCA also seems to affect the disease phenotype of ANCA-associated vasculitis. Two types of ANCAs are of clinical significance in patients with vasculitis:
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تاریخ انتشار 2013