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Initial presentation of intestitial lung disease and anti-Jo-1 antibodies: difference between acute and gradual-onset
  1. Tillie-Leblond Isabelle (i-tillie{at}chru-lille.fr)
  1. University Hospital of Lille, France
    1. Wislez Marie
    1. Lung department, Hopital Tenon, Paris, France
      1. Valeyre Dominique
      1. Lung department, Hopital Avicenne, Paris, France
        1. Crestani Bruno
        1. Lung department, Hopital Bichat, Paris, France
          1. Rabbat Antoine
          1. Lung department, Hopital Hotel-Dieu, Paris, France
            1. Israel-Biet Dominique
            1. Lung department, Hopital Europeen Pompidou, Paris, France
              1. Humbert Marc
              1. Lung department, Hopital Béclère, clamart, France
                1. Couderc Louis Jean
                1. Lung department, Hopital Foch, Suresnes, France
                  1. Wallaert Benoit
                  1. Lung department, Hopital Calmette, Lille, France
                    1. Cadranel Jacques (jacques.cadranel{at}tnn.ap-hop-paris.fr)
                    1. Lung department, Hopital Tenon, Paris, France

                      Abstract

                      The aims of this multicentre retrospective study were to collect and examine cases of patients with interstitial lung disease (ILD) as the initial clinical manifestation of an anti-synthetase syndrome (anti-Jo-1 antibodies), and to analyse the characteristics and long-term outcome of these patients according to their clinical presentation (acute or gradual onset), treatment and adverse events related to treatment. We describe 32 patients, 15 (47%) presenting with acute onset and associated respiratory insufficiency (Group A), and 17 (53%) with gradual onset (Group G). Myositis was diagnosed at admission in only 31% of cases, and was observed during follow-up in 56% of cases, but frequency did not differ between the two groups. Fever and radiological patterns associating diffuse patchy ground-glass opacities, basal irregular lines and consolidations on high-resolution CT scan were more frequent in Group A than in Group G (p=0.01). In Group G, most patients had neutrophils in bronchioloalveolar lavage, and autoantibodies other than anti-Jo-1 (rheumatoid factor, anti SSa/SSb) compared with Group A. The percentage of patients displaying improvement in ILD at three months was significantly higher in Group A (13/15) than in Group G (9/17), (p=0.006). In contrast, after 12 months, most patients displaying ILD progression were in Group A and were treated with corticosteroids alone. A combination of corticosteroids and and an immunosuppressive drug was required in most cases (84%) at the end of the follow-up period. Severe adverse effects of treatment were observed and varicella zoster virus infection was frequent. Early testing for anti-synthetase antibodies, particularly anti-Jo-1, and creatine kinase determination are useful procedures in patients presenting with ILD. Treatment with corticosteroids and immunosuppressive drug is required in most patients for ILD. At the end of the study, around two-thirds of patients had stable ILD, whereas the other third displayed progression with respiratory insufficiency.

                      • JO-1
                      • interstitial pneumonitis
                      • myositis

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