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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
Show Affiliations
Authors Affiliations
  1. 1. Department of Rheumatology, Universite Paris Cite, Cochin Hospital, Paris, France
  2. 2. Instituto Portugues de Reumatologia, Lisboa, Portugal
  3. 3. Department of Rheumatology, Martina Hansen Hospital, Sandvika, Norway
  4. 4. Department of Rheumatology, Oslo University Hospital, Oslo, Norway
  5. 5. Department of Internal Medicine and Clinical Immunology, Groupe Hospitalier Pitie-Salpetriere, AP-HP, Paris, F-75013, France
  6. 6. Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UNIRAR), Irccs San Raffaele Hospital, Milan, Italy
  7. 7. Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy
  8. 8. Servico de Reumatologia e Doencas Osseas Metabolicas, Centro Hospitalar Universitario Lisboa Norte and Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Centro Academico de Medicina de Lisboa (CAML), Lisbon, Portugal
  9. 9. Servico de Reumatologia, Hospital Garcia de Orta, Almada, Portugal
  10. 10. Department of Clinical, Internal, Anaesthesiologic, Cardiologic Sciences, University of Rome Sapienza, Rome, Italy
  11. 11. Department of Internal Medicine and Clinical Immunology, Rennes University Hospital, Rennes, France
  12. 12. Department of Rheumatology and Systemic Autoimmune Diseases, Hospital Universitari de la Santa Creu i Sant Pau, Barcelona, Spain
  13. 13. Rheumatology and Clinical Immunology Unit, Spedali Civili, Brescia, Italy
  14. 14. Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan
  15. 15. Rheumatology Research Center, Shariati Hospital, Tehran University of Medical Sciences, Kargar Avenue, 14117-13137, Tehran, Iran
  16. 16. INSERM U1016, Institut Cochin, CNRS UMR8104, Paris, France

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