Good outcome of interstitial lung disease in patients with scleroderma associated to anti-PM/Scl antibody

Alfredo Guillen-Del Castillo, Carmen Pilar Simeón-Aznar, Vicent Fonollosa-Pla, Serafín Alonso-Vila, María M. Reverte-Vinaixa, Xavier Muñoz, Esther Pallisa, Albert Selva-O'Callaghan, Andreu Fernández-Codina, Miquel Vilardell-Tarrés

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32 Citations (Scopus)


© 2014 Elsevier Inc. Objective: The objective of this article was to establish the clinical course of interstitial lung disease (ILD) in scleroderma related to the presence of anti-PM/Scl antibody compared with anti-Scl-70 in a Spanish cohort. Furthermore, no study has thoroughly investigated the outcome of pulmonary function test in the first group of patients. Methods: A total of 63 Spanish patients with scleroderma and ILD were selected in a retrospective observational study. Among them, 14 were positive for anti-PM/Scl antibodies and 49 for anti-Scl-70. Clinical assessments, including pulmonary function test, were collected. Variations equal or greater than 10% in forced vital capacity (FVC) were considered significant. Progression-free survival of disease was defined as the period of stable illness since pulmonary fibrosis diagnosis. Results: Anti-Scl-70 patients had a higher frequency of diffuse SSc subset, peripheral vasculopathy, and gastrointestinal involvement. Inflammatory myopathy was associated to anti-PM/Scl antibody. Anti-PM/Scl patients presented more improvement in FVC during follow-up, 30.8% compared to a 7.1% in Scl-70 group (. P = 0.04), with less worsening of this parameter (15.4% vs 52.4% in Scl-70 patients, P = 0.01), and secondary less frequency of severe restrictive pattern (FVC < 50%) (7.7% compared to 42.9% in the other group, P = 0.02). Regarding treatment, more anticalcineurinics were used in anti-PM/Scl patients, while cyclophosphamide and mycophenolate were mainly used in anti-Scl-70 patients. The progression-free survival of disease was higher in anti-PM/Scl patients, with 76% at 10 years from diagnosis of ILD against a 29% in the Scl-70 group. Conclusions: Several features and prognosis of ILD in SSc may be modified depending on the identified immunological profile.
Original languageEnglish
Pages (from-to)331-337
JournalSeminars in Arthritis and Rheumatism
Issue number3
Publication statusPublished - 1 Jan 2014


  • Anti-PM/Scl antibodies
  • Anti-topoisomerase I antibodies
  • Interstitial lung disease
  • Prognosis
  • Pulmonary fibrosis
  • Pulmonary function test
  • Systemic sclerosis


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