Microscopic polyangiitis

Microscopic polyangiitis or polyarteritis is a separate entity that should be distinguished from polyarteritis nodosa and Wegener's granulomatosis. Microscopic polyangiitis is characterized by pauci-immune necrotizing and crescentic glomerulonephritis and other systemic involvement in the absence of granulomas. Recurrent and diffuse alveolar hemorrhage due to pulmonary capillaritis is the main pulmonary feature of the disease. The presence of perinuclear type ANCA with specificity for myeloperoxidase (MPO-ANCA) is recognized as an indicator of the disease, although it is positive in only 60 per cent of patients. Clinically, microscopic polyangiitis can be confused with Wegener's granulomatosis because of hemoptysis, alveolar hemorrhage, and the presence of c-ANCA in about 40 per cent of patients. Acute respiratory failure caused by pulmonary hemorrhage, acute interstitial processes, and the acute respiratory distress syndrome have been described in this syndrome. Pathologically, interstitial inflammatory cells (alveolitis), hyaline membranes, restructuring, and fibrosis have been observed. The upper airway complications seen in Wegener's granulomatosis are conspicuously absent in microscopic polyangiitis, although progressive airway obstruction has been reported. The treatment of microscopic polyangiitis is similar to that of Wegener's granulomatosis.

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