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.
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