Page 267 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

In: The Vasculitides, Volume 1 ISBN: 978-1-63463-110-5
Editors: David S. Younger © 2015 Nova Science Publishers, Inc.

Chapter 13

Systemic Vasculitis and the Lung

Christian Pagnoux, M.D., M.P.H.?1 and Gerard P. Cox, M.D., M.B.2

1Mount Sinai Hospital, Division of Rheumatology – Vasculitis clinic,
Toronto, Ontario, Canada

2Division of Respirology, McMaster University, St. Joseph's Healthcare,
Hamilton, Ontario, Canada

Abstract

The lungs are affected by systemic vasculitides with differing frequency,
characteristics, and severity, depending on the specific vasculitis syndrome. Alveolar
hemorrhage, nodules, interstitial disease and airway stenoses are the most common lung
manifestations of antineutrophil cytoplasm antibody-associated vasculitides. Pulmonary
vascular involvement also occurs in Behçet disease and Takayasu arteritis, where it
manifests as pulmonary artery aneurysm, thrombosis and stenoses. Lung involvement
usually occurs along with other systemic features of the vasculitis syndrome but can also
be isolated leading to diagnostic and therapeutic challenges. Lung infection, pulmonary
edema due to cardiac failure or renal impairment, cancer, and drug-induced lung toxicity
are potential mimickers of pulmonary involvement in vasculitis. The management of lung
disease in patients with vasculitis includes systemic and local therapies.

Introduction

The lungs are affected by systemic vasculitides with differing frequency, characteristics
and severity, which vary by type of vasculitis in terms of involvement of the airways, lung
parenchyma, intraparenchymal proximal pulmonary arteries and veins, small capillary vessels
and pleura. These lung manifestations can be clinically silent or life-threatening, as with
diffuse alveolar hemorrhage. They can occur with other systemic manifestations of vasculitis

? Corresponding Author: Dr. Christian Pagnoux, Mount Sinai Hospital, Division of Rheumatology – Vasculitis
clinic, Room 2-220, 60 Murray Street, Toronto, ON, M5T 3L9, Canada. Email: [email protected]

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