Thorax 2000;55:502-510 ( June )

Review series

Rare diseases  10

Small vessel vasculitis of the lung

Marvin I Schwarz, Kevin K Brown

Interstitial Lung Disease Center, Division of Pulmonary Sciences and Critical Care Medicine, National Jewish Medical and Research Center and University of Colorado Health Sciences Center, Denver, Colorado 80262, USA

Correspondence to: Dr M I Schwarz email: marvin.schwarz@uchsc.edu

The first 150 words of the full text of this article appear below.

	Definition and aetiologies

The small vessel vasculitides of the lungs^1-3 (table 1) are inflammatory destructive processes that affect the arterioles, venules, and alveolar capillaries located within the interstitial compartment. An intense infiltration of activated neutrophils results in fibrinoid necrosis and dissolution of arteriolar and venular walls, thus compromising the vascular lumen. Accompanying the arteriolitis and venulitis is a distinct interstitial (alveolar wall) component referred to as necrotising pulmonary capillaritis.^4-9 Necrotising pulmonary capillaritis can occur in isolation, in the absence of histological evidence of arteriolitis or venulitis. It is recognised by a marked interstitial neutrophilic infiltration and many of these cells are undergoing leucocytoclasis or fragmentation (fig 1). Because these neutrophils are constantly undergoing cell death (apoptosis), pyknotic cells and nuclear fragments (dust) accumulate within the lung parenchyma. The interstitial space becomes broadened by oedema, fibrin, and the neutrophilic infiltrate and eventually undergoes fibrinoid necrosis (fig 2). During this process the . . . [Full text of this article]

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