PDF icon Download PDF
Semin Respir Crit Care Med 2018; 39(04): 465-470
DOI: 10.1055/s-0038-1669914
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Antineutrophil Cytoplasmic Antibody–Associated Lung Fibrosis

Raphael Borie
1   Assistance Publique Hôpitaux de Paris, Bichat Hospital, Rare Pulmonary Diseases Reference Center, Paris, France
2   Institut National de la Recherche Médicale, Unit 1152, DHU FIRE, Paris Diderot University, Paris, France
,
Bruno Crestani
1   Assistance Publique Hôpitaux de Paris, Bichat Hospital, Rare Pulmonary Diseases Reference Center, Paris, France
2   Institut National de la Recherche Médicale, Unit 1152, DHU FIRE, Paris Diderot University, Paris, France
› Author Affiliations
Further Information

Publication History

Publication Date:
07 November 2018 (online)

   Permissions and Reprints
Preview

Abstract

Pulmonary fibrosis is observed in a substantial number of patients with ANCA-associated vasculitis (AAV), 15% in a recent German series, and may be more frequent in Asian populations. ANCA are usually of anti-MPO specificity and microscopic polyangiitis is the most frequent vasculitis. Pulmonary fibrosis may increase the risk of death in patients with AAV. Treatment for AAV in patients with lung fibrosis should follow the international guidelines for vasculitis. The role of anti-fibrotic drugs (pirfenidone, nintedanib) in this condition is still unknown. Pulmonary fibrosis precedes the diagnosis of AAV or is diagnosed concomitantly in most of the cases. Interestingly, 4% to 35% of patients with pulmonary fibrosis are ANCA-positive, but only 7% to 23% of the patients with pulmonary fibrosis and anti-MPO will develop AAV during follow-up. ANCA positivity may be detected in idiopathic or non idiopathic pulmonary fibrosis. In the absence of vasculitis, the detection of ANCA does not influence the diagnostic work-up of patients with lung fibrosis. If an Idiopathic Pulmonary Fibrosis diagnosis is considered, an anti-fibrotic therapy should be considered, according to local and international guidelines.

Keywords

idiopathic pulmonary fibrosis - STING - COPA - usual interstitial pneumonia - microscopic polyangiitis - myeloperoxidase