Sjögren's disease & overlap diseases
The phenotype of mixed connective tissue disease patients having associated interstitial lung disease
Authors' 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.
Study context
- Population
- MCTD patients fulfilling at least one historical MCTD classification criteria, 14 tertiary hospitals (mean age at diagnosis 39.7 ± 15.4 years; 63.7% women)191 (63.7%) women
- Participants
- 300 patients
- Exposure
- MCTD with ILD vs MCTD without ILD
- Outcome
- Factors associated with ILD presence at baseline
- Follow-up
- Baseline
Methodological detailsinternational, multicentre (14 tertiary hospitals), matched populations
- International
- Multicentre (14 tertiary hospitals)
- Matched populations
Main finding
As reported in the abstract
- Older age
- p = 0.01
- Skin thickening
- p = 0.03
- Upper gastro-intestinal (GI) symptoms
- p<0.001
- Forced vital capacity (FVC) <80%
- p<0.0001
- DLCO <80%
- p<0.0001
- Anti-topoisomerase antibodies
- p = 0.01
- SSA/Ro antibodies
- p = 0.02
- Cryoglobulinemia
- p = 0.04
- Elevated CRP
- p<0.001
Observational data: associations, not effects.
Further findings
| Outcome | P value |
|---|---|
| Mortality during follow-up (higher in MCTD-ILD) · mean follow-up 7.8 ± 5.5 years | P < 0.001 |
Treatment with synthetic immunosuppressants (more likely in MCTD-ILD)
- Synthetic immunosuppressant agents
- P < 0.001
- MMF
- P = 0.03
Risk factor for FVC decline >10% (progressive ILD)
- Digital ulcers identified as a risk factor for FVC decline >10%
Original publication
The phenotype of mixed connective tissue disease patients having associated interstitial lung disease. Semin Arthritis Rheum. Published 26 August 2023. doi:10.
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