Pulmonary fibrosis associated with ANCA-positive vasculitides. Retrospective study of 12 cases and review of the literature

B Hervier ^1*, C Pagnoux ², C Agard ¹, J Haroche ³, Z Amoura ⁴, L Guillevin ⁵ and M A Hamidou ⁶

¹ CHU NANTES, Hotel Dieu, France
² Assistance Publique - Hôpitaux de Paris, Hôpital Cochin, France
³ Service de médecine interne, CHU Pitié-Salpetrière, Paris, France
⁴ Service de Médecine Interne, Hopital Pitié-Salpëtrière, Paris, France
⁵ Hopital Cochin, France
⁶ CHU Hôtel-Dieu. Nantes University Hospital, France

^* To whom correspondence should be addressed. E-mail: baptiste.hervier{at}chu-nantes.fr.

Accepted 29 September 2008

Abstract

Objective: Association of pulmonary fibrosis (PF) and anti-neutrophil cytoplasmic antibodies (ANCA)-related systemic vasculitis (ANCA-V) is rare. The aim of our study was to describe the clinical presentation of this association.

Methods: We retrospectively studied 12 patients with ANCA-V associated with "idiopathic" PF.

Results: There were 3 females and 9 males, and the mean age was 70.7 years. In 8 cases, ANCA-V and PF were diagnosed simultaneously; PF occured earlier in 3 cases, and during the ANCA-V follow-up in one. No patient had argument for intra-alveolar hemorrhage (IAH). ANCA were MPO-ANCAs (myeloperoxydase) in all cases. Seven patients had a blood eosinophilia at diagnosis. Two patients died during the ANCA-V induction therapy. Five patients had a respiratory status worsening and 3 of them died (later than 37 months after diagnosis) from exacerbation of end-stage respiratory failure. The five remaining patients had a respiratory status stability.

Conclusion: The association of PF and ANCA-V does not seem fortuitous, eventhough their clinical evolutions are clearly not related. PF was the major cause of death. ANCA-V has several characteristics, in accordance with the literature : generally male gender, older age, absence of IAH, frequent blood eosinophilia and ANCAs directed against MPO.