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Interstitial pneumonia with autoimmune features: Evaluation of connective tissue disease incidence during follow-up

  • Paul Decker
    Correspondence
    Corresponding author.
    Affiliations
    Department of Internal Medicine and Clinical Immunology, Center de Compétence des Maladies Autoimmunes Systémiques Rares, CHU Nancy, University of Lorraine, 5 Rue du Morvan, Vandoeuvre-lès-Nancy 54500, France
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  • Vincent Sobanski
    Affiliations
    Department of Internal Medicine and Clinical Immunology, Center de Référence des maladies Autoimmunes Systémiques Rares du Nord et Nord-Ouest de France (CeRAINO), CHU Lille, University of Lille, Lille F-59000, France

    University of Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, Lille F-59000, France

    INSERM, Lille F-59000, France

    Institut Universitaire de France (IUF), France
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  • Thomas Moulinet
    Affiliations
    Department of Internal Medicine and Clinical Immunology, Center de Compétence des Maladies Autoimmunes Systémiques Rares, CHU Nancy, University of Lorraine, 5 Rue du Morvan, Vandoeuvre-lès-Nancy 54500, France

    UMR 7365, IMoPA, University of Lorraine, CNRS, Nancy, France
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  • David Launay
    Affiliations
    Department of Internal Medicine and Clinical Immunology, Center de Référence des maladies Autoimmunes Systémiques Rares du Nord et Nord-Ouest de France (CeRAINO), CHU Lille, University of Lille, Lille F-59000, France

    University of Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, Lille F-59000, France

    INSERM, Lille F-59000, France
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  • Eric Hachulla
    Affiliations
    Department of Internal Medicine and Clinical Immunology, Center de Référence des maladies Autoimmunes Systémiques Rares du Nord et Nord-Ouest de France (CeRAINO), CHU Lille, University of Lille, Lille F-59000, France

    University of Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, Lille F-59000, France

    INSERM, Lille F-59000, France
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  • Victor Valentin
    Affiliations
    Department of Pneumology and Immuno-Allergology, Center de Référence Constitutif Pour les Maladies Pulmonaires Rares, CHU Lille, University of Lille, Lille F-59000, France
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  • Benoit Godbert
    Affiliations
    Department of Pneumology, Metz Private Hospital, Metz, France
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  • Sabine Revuz
    Affiliations
    Department of Internal Medicine, Metz Private Hospital, Metz, France
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  • Anne Guillaumot
    Affiliations
    Department of Pneumology, CHU Nancy, University of Lorraine, Vandoeuvre-lès-Nancy, France
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  • Emmanuel Gomez
    Affiliations
    Department of Pneumology, CHU Nancy, University of Lorraine, Vandoeuvre-lès-Nancy, France
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  • François Chabot
    Affiliations
    Department of Pneumology, CHU Nancy, University of Lorraine, Vandoeuvre-lès-Nancy, France
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  • Lidwine Wémeau
    Affiliations
    Department of Pneumology and Immuno-Allergology, Center de Référence Constitutif Pour les Maladies Pulmonaires Rares, CHU Lille, University of Lille, Lille F-59000, France
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  • Roland Jaussaud
    Affiliations
    Department of Internal Medicine and Clinical Immunology, Center de Compétence des Maladies Autoimmunes Systémiques Rares, CHU Nancy, University of Lorraine, 5 Rue du Morvan, Vandoeuvre-lès-Nancy 54500, France
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Published:December 22, 2021DOI:https://doi.org/10.1016/j.ejim.2021.12.021

      Highlights

      • Interstitial pneumonia with autoimmune features (IPAF) patients had more frequently a definite connective tissue disease (CTD) diagnosis during the disease course, especially antisynthetase syndrome and systemic sclerosis.
      • Identifying specific clinical and serological factors associated with CTD occurrence is essential at interstitial lung disease (ILD) diagnosis.

      Abstract

      Objectives

      Among interstitial pneumonia with autoimmune features (IPAF) patients, identifying those at risk to develop a connective tissue disease (CTD) during the disease course is a key issue. The aim of this study was to evaluate the incidence of definite CTD diagnosis in IPAF patients during follow-up.

      Methods

      We performed a multicentric cohort study of interstitial lung disease (ILD) from 2010 to 2017 in pneumology and immunology departments of tertiary care centers. Patients with a known cause of ILD (including established CTD) at diagnosis were excluded. Among patients with idiopathic ILD and at least three years of follow-up, two groups (IPAF and non-IPAF) were retrospectively analyzed at time of diagnosis.

      Results

      A total of 249 patients with ILD were enrolled, including 70 IPAF and 179 non-IPAF patients. After a mean follow-up time of 77 ± 44 months, 18/70 IPAF patients (26%) had a CTD diagnosis – 9 antisynthetase syndrome, 8 systemic sclerosis and 1 overlap myositis – compared with 4/179 non-IPAF patients (2%). IPAF patients were at higher risk of CTD occurrence at 3 years of follow-up compared to non-IPAF patients (HR 10.1, 95% CI 3.1–33.1, p < 0. 01). IPAF patients progressing to CTD tended to be younger, more often female and have more frequently puffy fingers, capillaroscopy abnormalities and antisynthetase antibodies at diagnosis.

      Conclusions

      We found that a significant proportion of IPAF patients had associated CTD diagnosis during follow-up. Prospective studies are needed to confirm baseline predictive factors of CTD occurrence in IPAF patients.

      Keywords

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