Longitudinal follow-up of mixed connective tissue disease and overlapping autoimmune diseases of childhood onset in the Afro-descendant population of the French West Indies
Authors' conclusion
The clinical evolution did not differ between MCTD and OAS. The main complication was PAH, more frequent in our cohort.
Study context
- Population
- Afro-Caribbean patients from the French West Indies with childhood-onset mixed connective tissue disease (MCTD) or overlap autoimmune syndromes, January 2000 to 202323-year period
- Participants
- 16 patients
- Exposure
- MCTD vs overlap autoimmune syndromes (OAS)
- MCTDn = 10
- OASn = 6
- Outcome
- Characteristics and outcomes during childhood
Methodological detailslongitudinal, descriptive, multiple-source case ascertainment
- Longitudinal
- Descriptive
- Multiple-source case ascertainment
Main finding
As reported in the abstract
- Incidence (per 100,000 children-years)
- 0.23
- Mean age at diagnosis, years (range 2.4-17)
- 11.9
- Median follow-up, years (range 2.1-19.6)
- 7.9
- Average number of flares during childhood (range 1-7)
- 3
- Symptomatic pulmonary arterial hypertension (PAH)
- 25%
- Received steroids during follow-up
- 94%
- Required a corticosteroid-sparing therapy
- 88%
- Developed systemic lupus erythematosus (SLE) after more than 10y of follow-up (19%)
- 3
Observational data: associations, not effects.
Further findings
- No death and no chronic organ failure
Original publication
Longitudinal follow-up of mixed connective tissue disease and overlapping autoimmune diseases of childhood onset in the Afro-descendant population of the French West Indies. Pediatr Rheumatol Online J. Published 11 January 2024. doi:10.
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