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1: Ann Rheum Dis. 2008 Oct 28. [Epub ahead of print]
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Pulmonary fibrosis associated with ANCA-positive vasculitides. Retrospective study of 12 cases and review of the literature.

Hervier B, Pagnoux C, Agard C, Haroche J, Amoura Z, Guillevin L, Hamidou MA.

CHU NANTES, Hotel Dieu, France.

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.

PMID: 18957485 [PubMed - as supplied by publisher]