Pulmonary vasculitis

Abstract

Pulmonary vasculitis describes a number of distinct disorders that are pathologically characterized by the destruction of blood vessels. The clinical manifestations of each disorder are defined by the size, type, and location of the affected vasculature. The clinical approach to these disorders rests upon an astute clinician considering the diagnosis and identifying the specific patterns of clinical, radiologic, laboratory, and pathologic abnormalities. Lung involvement is most commonly seen with the primary, idiopathic, small-vessel, or antineutrophil cytoplasmic antibody-associated vasculitides; Wegener's granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome. However, primary, idiopathic medium and large-vessel vasculitis, primary immune complex-mediated vasculitis, and secondary vasculitis are all capable of presenting with lung involvement. In this article, we focus on the more common, antineutrophil cytoplasmic antibody-associated disorder, vasculitides.

Figure 1.

(A) High-resolution computed tomographic image of diffuse alveolar hemorrhage. The general pattern is that of patchy ground glass attenuation with or without consolidation. (B) On surgical lung biopsy, the histopathology of pulmonary capillaritis notes infiltration and expansion of the alveolar septae by degenerating neutrophils (arrow). Radiographic image courtesy of David A. Lynch, M.D.; pathologic image courtesy of Carlyne D. Cool, M.D.

Figure 2.

Indirect immunofluorescence staining for anti- neutrophil cytoplasmic antibodies (ANCA). (A) The granular cytoplasmic staining pattern of C-ANCA. (B) The perinuclear pattern of P-ANCA. Images courtesy of Ron Harbeck, Ph.D.

Figure 3.

Histopathology of Wegener's granulomatosis. (A) With vasculitis identified by eccentric inflammation (arrow) of a medium-sized artery with partial destruction of the elastic laminae on pentachrome staining. (B) With destructive vasculitis (arrow identifies remnants of elastic laminae). The artery is otherwise destroyed by an inflammatory process. (C) With granulomatous inflammation with a multinucleated giant cell (arrow). (D) With irregular, geographic necrosis with basophilic staining (arrow). Images courtesy of Carlyne D. Cool, M.D.

Figure 4.

Survival curve of treated vasculitis. Reprinted by permission from Reference 54.