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10.1097/MD.0000000000000217

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C4602438!4602438 !25500703
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suck abstract from ncbi


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pmid25500703
      Medicine+(Baltimore) 2014 ; 93 (24 ): 340-349
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  • Pulmonary fibrosis in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis: a series of 49 patients and review of the literature #MMPMID25500703
  • Comarmond C ; Crestani B ; Tazi A ; Hervier B ; Adam-Marchand S ; Nunes H ; Cohen-Aubart F ; Wislez M ; Cadranel J ; Housset B ; Lloret-Linares C ; Sève P ; Pagnoux C ; Abad S ; Camuset J ; Bienvenu B ; Duruisseaux M ; Hachulla E ; Arlet JB ; Hamidou M ; Mahr A ; Resche-Rigon M ; Brun AL ; Grenier P ; Cacoub P ; Saadoun D
  • Medicine (Baltimore) 2014[Nov]; 93 (24 ): 340-349 PMID25500703 show ga
  • Pulmonary fibrosis (PF) is an uncommon manifestation observed in patients with antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV), particularly microscopic polyangiitis (MPA). While patients with PF associated with AAV seem to have a worse prognosis, these patients have been described only in case reports or small retrospective case series. In this retrospective multicenter study, we report the main features and long-term outcomes of patients with PF associated with AAV, fulfilling the American College of Rheumatology criteria and/or Chapel Hill definitions. Forty-nine patients (30 men [61%]; median age at diagnosis of AAV, 68 [interquartile range, 58-73] years) with PF associated with AAV were identified. Forty (81.6%) patients had MPA and 9 (18.4%) had granulomatosis with polyangiitis. The diagnosis of PF preceded the onset of vasculitis in 22 (45%) patients. Usual interstitial pneumonia was the main radiologic pattern (n?=?18, 43%). ANCA were mostly of antimyeloperoxidase specificity (88%). All patients were treated with glucocorticoids as induction therapy, combined with cyclophosphamide (CYC) (n?=?36, 73.5%) or rituximab (RTX) (n?=?1, 2%). Factors associated with mortality included occurrence of chronic respiratory insufficiency (hazard ratio [HR], 7.44; 95% confidence interval [CI], 1.6-34.5; p?=?0.003), induction therapy with glucocorticoids alone (HR, 2.94; CI, 1.05-8.33; p?=?0.04), and initial weigh loss (HR, 2.83; CI, 1.05-7.65; p?=?0.041). The 3-year survival rate in patients treated with glucocorticoids alone or combined with an immunosuppressant (CYC or RTX) as induction therapy was 64% (95% CI, 41-99) and 94% (95% CI, 86-100), respectively (p?=?0.03). After a median follow-up of 48 months [interquartile range, 14-88 mo], 18 (37%) patients died, including 11 related to respiratory insufficiency. PF is a rare manifestation of AAV with a very poor prognosis. Induction therapy with CYC might improve the outcome.
  • |Aged [MESH]
  • |Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/*complications/diagnosis [MESH]
  • |Bronchoalveolar Lavage [MESH]
  • |Female [MESH]
  • |Humans [MESH]
  • |Male [MESH]
  • |Middle Aged [MESH]
  • |Prognosis [MESH]
  • |Pulmonary Fibrosis/diagnostic imaging/*etiology/physiopathology [MESH]
  • |Respiratory Function Tests [MESH]
  • |Retrospective Studies [MESH]


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