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The Phenotype of Mixed Connective Tissue Disease Patients Having Associated Interstitial Lung Disease Publisher Pubmed



Boleto G1, 2 ; Reiseter S3 ; Hoffmannvold AM4 ; Mirouse A5 ; Cacoub P5 ; Matuccicerinic M6, 7 ; Silverioantonio M8 ; Fonseca JE8 ; Duarte AC9 ; Pestana Lopes J9 ; Riccieri V10 ; Lescoat A11 ; Le Tallec E11 ; Castellvi Barranco I12 Show All Authors
Authors
  1. Boleto G1, 2
  2. Reiseter S3
  3. Hoffmannvold AM4
  4. Mirouse A5
  5. Cacoub P5
  6. Matuccicerinic M6, 7
  7. Silverioantonio M8
  8. Fonseca JE8
  9. Duarte AC9
  10. Pestana Lopes J9
  11. Riccieri V10
  12. Lescoat A11
  13. Le Tallec E11
  14. Castellvi Barranco I12
  15. Tandaipan JL12
  16. Airo P13
  17. Kuwana M14
  18. Kavosi H15
  19. Avouac J1, 16
  20. Allanore Y1, 16

Source: Seminars in Arthritis and Rheumatism Published:2023


Abstract

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. © 2023