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Semin Respir Crit Care Med 2018; 39(04): 504-510
DOI: 10.1055/s-0038-1669414
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Treatment of Pulmonary Vasculitis

Benjamin Terrier
1   Department of Internal Medicine, National Referral Center for Rare Systemic and Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris Descartes, Paris, France
,
Loïc Guillevin
2   Department of Internal Medicine, Hôpital Cochin, Université Paris, Paris, France
› Author Affiliations
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Publication History

Publication Date:
07 November 2018 (online)

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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.

Keywords

glucocorticoids - cyclophosphamide - rituximab - plasma exchanges - vasculitis - pulmonary vasculitis
 

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