Article Dans Une Revue Seminars in Arthritis and Rheumatism Année : 2023

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

Gonçalo Boleto
Silje Reiseter
  • Fonction : Auteur
Valeria Riccieri
  • Fonction : Auteur
Jérôme Avouac

Résumé

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.
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Dates et versions

hal-04244332 , version 1 (04-12-2023)

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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. ⟨10.1016/j.semarthrit.2023.152258⟩. ⟨hal-04244332⟩
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