Pulmonary manifestations of large, medium, and variable vessel vasculitis

Respir Med. 2018 Dec:145:182-191. doi: 10.1016/j.rmed.2018.11.003. Epub 2018 Nov 10.

Abstract

The hallmark of vasculitis is autoimmune inflammation of blood vessels and surrounding tissues, resulting in an array of constitutional symptoms and organ damage. The lung is commonly targeted in the more familiar ANCA-associated small vessel vasculitidies, but large and medium vessel vasculitides, including Takayasu arteritis, giant cell arteritis, polyarteritis nodosa, Behcet's disease, and necrotizing sarcoid granulomatosis, may also feature prominent pulmonary involvement. Pulmonary manifestations of these conditions include pulmonary arterial aneurysms, pulmonary hypertension, diffuse alveolar hemorrhage, pulmonary nodules, and parenchymal infiltrates. An understanding of the diverse manifestations of vasculitis and a high index of clinical suspicion are essential to avoid delays in disease recognition that may result in permanent or life threatening morbidity. In this review, we outline the general clinical manifestations, pulmonary manifestations, diagnostic workup, imaging findings, and treatment of medium, large, and variable vessel vasculitides.

Keywords: Aneurysm; Arteritis; Vasculitis.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Aneurysm / etiology*
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / complications*
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / diagnosis
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / therapy
  • Behcet Syndrome / complications*
  • Behcet Syndrome / diagnosis
  • Behcet Syndrome / therapy
  • Giant Cell Arteritis / complications*
  • Giant Cell Arteritis / diagnosis
  • Giant Cell Arteritis / therapy
  • Hemorrhage / etiology*
  • Humans
  • Hypertension, Pulmonary / etiology*
  • Lung Diseases / etiology*
  • Polyarteritis Nodosa / complications*
  • Polyarteritis Nodosa / diagnosis
  • Polyarteritis Nodosa / therapy
  • Pulmonary Alveoli*
  • Pulmonary Artery*
  • Takayasu Arteritis / complications*
  • Takayasu Arteritis / diagnosis
  • Takayasu Arteritis / therapy