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

Autor: Boleto G; Department of Rheumatology, Université Paris Cité, Cochin Hospital, Paris, France; Instituto Português de Reumatologia, Lisboa, Portugal., Reiseter S; Department of Rheumatology, Martina Hansen Hospital, Sandvika, Norway., Hoffmann-Vold AM; Department of Rheumatology, Oslo University Hospital, Oslo, Norway., Mirouse A; Department of Internal Medicine and Clinical Immunology, Groupe Hospitalier Pitié-Salpêtrière, AP-HP, Paris F-75013, France., Cacoub P; Department of Internal Medicine and Clinical Immunology, Groupe Hospitalier Pitié-Salpêtrière, AP-HP, Paris F-75013, France., Matucci-Cerinic M; Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UNIRAR), Irccs San Raffaele Hospital, Milan, Italy; Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy., Silvério-António M; Serviço de Reumatologia e Doenças Ósseas Metabólicas, Centro Hospitalar Universitário Lisboa Norte and Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa (CAML), Lisbon, Portugal., Fonseca JE; Serviço de Reumatologia e Doenças Ósseas Metabólicas, Centro Hospitalar Universitário Lisboa Norte and Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa (CAML), Lisbon, Portugal., Duarte AC; Serviço de Reumatologia, Hospital Garcia de Orta, Almada, Portugal., Pestana Lopes J; Serviço de Reumatologia, Hospital Garcia de Orta, Almada, Portugal., Riccieri V; Department of Clinical, Internal, Anaesthesiologic, Cardiologic Sciences, University of Rome Sapienza, Rome, Italy., Lescoat A; Department of Internal Medicine and Clinical Immunology, Rennes University Hospital, Rennes, France., Le Tallec E; Department of Internal Medicine and Clinical Immunology, Rennes University Hospital, Rennes, France., Castellví Barranco I; Department of Rheumatology and Systemic Autoimmune Diseases, Hospital Universitari de la Santa Creu i Sant Pau, Barcelona, Spain., Tandaipan JL; Department of Rheumatology and Systemic Autoimmune Diseases, Hospital Universitari de la Santa Creu i Sant Pau, Barcelona, Spain., Airó P; Rheumatology and Clinical Immunology Unit, Spedali Civili, Brescia, Italy., Kuwana M; Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan., Kavosi H; Rheumatology Research Center, Shariati Hospital, Tehran University of Medical Sciences, Kargar Avenue, 14117-13137, Tehran, Iran., Avouac J; Department of Rheumatology, Université Paris Cité, Cochin Hospital, Paris, France; INSERM U1016, Institut Cochin, CNRS UMR8104, Paris, France., Allanore Y; Department of Rheumatology, Université Paris Cité, Cochin Hospital, Paris, France; INSERM U1016, Institut Cochin, CNRS UMR8104, Paris, France. Electronic address: yannick.allanore@aphp.fr.
Jazyk: angličtina
Zdroj: Seminars in arthritis and rheumatism [Semin Arthritis Rheum] 2023 Dec; Vol. 63, pp. 152258. Date of Electronic Publication: 2023 Aug 26.
DOI: 10.1016/j.semarthrit.2023.152258
Abstrakt: Objective: We 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.
Methods: This 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.
Results: 300 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).
Conclusion: In 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.
Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
(Copyright © 2023. Published by Elsevier Inc.)
Databáze: MEDLINE
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