• Eur. J. Intern. Med. · Mar 2022

    Multicenter Study

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

    • Paul Decker, Vincent Sobanski, Thomas Moulinet, David Launay, Eric Hachulla, Victor Valentin, Benoit Godbert, Sabine Revuz, Anne Guillaumot, Emmanuel Gomez, François Chabot, Lidwine Wémeau, and Roland Jaussaud.
    • 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. Electronic address: p.decker@chru-nancy.fr.
    • Eur. J. Intern. Med. 2022 Mar 1; 97: 62-68.

    ObjectivesAmong 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.MethodsWe 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.ResultsA 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.ConclusionsWe 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.Copyright © 2021 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.

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