cover image: The phenotype of mixed connective tissue disease patients having associated interstitial lung disease

20.500.12592/4nyz6it

The phenotype of mixed connective tissue disease patients having associated interstitial lung disease

1 Dec 2023

ObjectiveWe aimed to compare two matched populations of patients with MTCD with and without associated ILD and to identify predictive factors for ILD progression and severity.MethodsThis international multicenter retrospective study (14 tertiary hospitals), included MCTD patients who fulfilled at least one historical MCTD classification criteria. ILD was defined by the presence of typical chest high-resolution computed tomography (HRCT) abnormalities. Factors associated with ILD were assessed at baseline. Long-term progressive ILD was assessed in MCTD-ILD patients with multiple forced vital capacity (FVC) measurements.Results300 patients with MCTD were included. Mean age at diagnosis was 39.7 ± 15.4 years and 191 (63.7%) were women. Mean follow-up was 7.8 ± 5.5 years. At baseline, we identified several factors associated with ILD presence: older age (p = 0.01), skin thickening (p = 0.03), upper gastro-intestinal (GI) symptoms (p<0.001), FVC <80% (p<0.0001), diffusing capacity for carbon monoxide <80% (p<0.0001), anti-topoisomerase antibodies (p = 0.01), SSA/Ro antibodies (p = 0.02), cryoglobulinemia (p = 0.04) and elevated C-reactive protein (p<0.001). Patients with MTCD-ILD were more likely to be treated with synthetic immunosuppressant agents (p<0.001) in particular mycophenolate mofetil (p = 0.03). Digital ulcers (DU) were identified as a risk factor for FVC decline >10%. During follow-up mortality was higher in the MTCD-ILD group (p<0.001).ConclusionIn this large international cohort of patients with MTCD, we identified different factors associated with ILD. Our findings also provide evidence that MCTD-ILD patients have increased mortality and that DU are associated with progressive lung disease.

Authors

Gonçalo Boleto, Silje Reiseter, Anna-Maria Hoffmann-Vold, Adrien Mirouse, Patrice Cacoub, Marco Matucci-Cerinic, Manuel Silvério-António, Joao Eurico Fonseca, Ana Catarina Duarte, Jorge Pestana Lopes, Valeria Riccieri, Alain Lescoat, Erwan Le Tallec, Ivan Castellví Barranco, Jose Luis Tandaipan, Paolo Airó, Masataka Kuwana, Hoda Kavosi, Jérôme Avouac, Yannick Allanore

Bibliographic Reference
Gonçalo Boleto, Silje Reiseter, Anna-Maria Hoffmann-Vold, Adrien Mirouse, Patrice Cacoub, et al.. The phenotype of mixed connective tissue disease patients having associated interstitial lung disease. Seminars in Arthritis and Rheumatism, 2023, 63, pp.152258. &#x27E8;10.1016/j.semarthrit.2023.152258&#x27E9;. &#x27E8;hal-04244332&#x27E9;
DOI
https://doi.org/10.1016/j.semarthrit.2023.152258
HAL Collection
['Université de Rennes 1', 'CNRS - Centre national de la recherche scientifique', "Université d'Angers", 'AP-HP', 'SFR Biosit - Biologie et Santé CNRS UMS 3480', 'Publications CHU - Rennes 1', 'Institut de Recherche en Santé, Environnement et Travail', 'TRANSCRIPTION, ENVIRONMENT and CANCER (TREC)', 'NEUROENDOCRINE EFFECTS OF ENDOCRINE DISRUPTORS', "Recherches épidémiologiques sur l'environnement, la reproduction et le développement", "UFR SVE Sciences de la vie et de l'environnement", 'Publications labos UR1 dans HAL-Rennes 1', 'UR1 - publications SDV', 'TEST Université de Rennes CSS', 'Université de Rennes', 'Sorbonne Université', 'Sorbonne Université 01/01/2018', 'Faculté de Santé de Sorbonne Université', 'Médecine - Sorbonne Université', 'Université Paris Cité', 'Université de Paris - Faculté de Santé', 'Sorbonne Université - Texte Intégral', 'Pôle Rennes 1 - Biologie-Santé', 'Alliance Sorbonne Université']
HAL Identifier
4321578
Institution
['Institut National de la Santé et de la Recherche Médicale', 'Université Paris Cité', 'Hôpital Cochin [AP-HP]', 'Oslo University Hospital [Oslo]', 'CHU Pitié-Salpêtrière [AP-HP]', 'Università degli Studi di Firenze = University of Florence', 'Universidade de Lisboa', 'Hospital Garcia de Orta', "Université d'Angers", 'Université de Rennes', 'École des Hautes Études en Santé Publique [EHESP]', 'Structure Fédérative de Recherche en Biologie et Santé de Rennes', 'Centre Hospitalier Universitaire de Rennes [CHU Rennes] = Rennes University Hospital [Ponchaillou]', 'Hospital de la Santa Creu i Sant Pau', 'Civic Hospital of Brescia', 'Nippon Medical School [Tokyo, Japon]', 'Tehran University of Medical Sciences']
Laboratory
['Institut Cochin', 'Institut de recherche en santé, environnement et travail']
Published in
France

Table of Contents