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Pulmonary Vasculitides

A Review

Callahan, Sean J., MD*; Sturek, Jeffrey M., MD, PhD; Richard, Ryan P., MD

doi: 10.1097/CPM.0000000000000290
Interstitial, Inflammatory & Occupational Lung Disease

Vasculitides are uncommon diseases that target multiple sites of the respiratory tract. Their manifestations vary widely, ranging from the banal (cough, rash) to life threatening (renal failure, diffuse alveolar hemorrhage). Small-vessel, antineutrophil cytoplasmic antibody–associated vasculitides are the principal forms affecting the lungs. Less often, larger vessel and immune-complex vasculitides afflict the respiratory tract, although the management of these conditions often mirrors that of the antineutrophil cytoplasmic antibody–associated vasculitides with immunosuppression and supplemental modalities such as plasma exchange. This paper reviews the epidemiology and clinical presentation of vasculitides with pulmonary involvement. The review also includes a pragmatic, evidence-based approach to management driven by disease severity.

*Division of Pulmonary and Critical Care, University of Utah Health, Salt Lake City, UT

Department of Medicine, Division of Pulmonary and Critical Care, University of Virginia School of Medicine, Charlottesville, VA

S.J.C. takes primary responsibility for all aspects of the manuscript.

Disclosure: The authors declare that they have no conflicts of interest.

Address correspondence to: Sean J. Callahan, MD, 26 N 1900 E, #701 Wintrobe, Salt Lake City, UT 84132. E-mail:

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