Treatment of Pulmonary Vasculitis

Benjamin Terrier, MD, PhD; Loïc Guillevin, MD

Disclosures

Semin Respir Crit Care Med. 2018;39(4):504-510. 

In This Article

Abstract and Introduction

Abstract

Pulmonary vasculitis can be heterogeneous and have some pathophysiological, clinical, and biological specificities that might influence treatment choices. Main vasculitides associated with pulmonary vasculitis include antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis, immune complex small vessel vasculitis, and antiglomerular basement membrane disease. Patient characteristics and association of lung involvement with other manifestations affecting disease severity may also influence treatment strategy. Alveolar hemorrhage frequently requires urgent treatment to control the underlying disease and provide supportive care such as mechanical ventilation. The level of evidence of each treatment strategy varies according to the underlying disease, but treatment is usually based on a combination of glucocorticoids and immunosuppressive agents, especially cyclophosphamide and rituximab. The role of plasma exchanges, widely used in patients with severe vasculitis, is controversial, based on results from recent prospective randomized controlled trials.

Introduction

Pulmonary vasculitis can be heterogeneous and have pathophysiological, clinical, and biological specificities that might influence treatment choices. Also, patient characteristics and association of lung involvement with other manifestations affecting disease severity should influence treatment strategy. Prognostic factors can be identified in some vasculitides to adapt therapeutic strategy. In this article, we focus on the therapeutic management of pulmonary vasculitis according to the main diseases.

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