Juvenile-onset mixed connective tissue disease: A multicenter retrospective cohort study
Authors' conclusion
jMCTD share the same clinical characteristics as aMCTD patients, but less frequently have puffy fingers. Outcomes appear more favorable in jMCTD than aMCTD, with higher remission rates, albeit at the cost of more intensive treatment.
Study context
- Population
- Patients with MCTD in the French MCTD cohort47 jMCTD
- Comparison
- Juvenile-onset MCTD vs matched adult-onset MCTD
- jMCTDn = 47
- Outcome
- Clinical features, treatments and outcomes of jMCTD vs aMCTD
Methodological detailsmulticentre, matched 1:3
- Multicentre
- Matched 1:3
Main finding
As reported in the abstract
Remission · median follow-up 9.8 [6.6-16.2] years
P < 0.05
Observational data: associations, not effects.
Further findings
Initial GC dose
P = 0.02
Progression to another CTD (mainly SLE) · median follow-up 9.8 [6.6-16.2] years
P = 0.5
- Puffy fingers · less frequent in jMCTD
- P < 0.0001
- MTX and RTX use over time · significantly more frequent in jMCTD
- P = 0.01
- Death in jMCTD · median follow-up 9.8 [6.6-16.2] years
- 2.1 % · 1 of 47
Cumulative treatment in jMCTD
%
- GC
- 80.9
- HCQ
- 95.7
- Immunosuppressants
- 93.6
ILD and PAH in jMCTD · median follow-up 9.8 [6.6-16.2] years
- ILD
- 11 (23.4 %)
- PAH
- 2 (4.3 %)
Original publication
Juvenile-onset mixed connective tissue disease: A multicenter retrospective cohort study. Semin Arthritis Rheum. Published 11 December 2025. doi:10.
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