Severe Features of Systemic Juvenile Idiopathic Arthritis in Patients With Congenital Heart Disease
Authors' conclusion
We identified an association between CHD and severe forms of sJIA.
Study context
- Population
- Patients diagnosed with congenital heart disease (CHD) who subsequently developed systemic juvenile idiopathic arthritis (sJIA), at the authors' institution
- Participants
- 5 patients
- Outcome
- Clinical features of sJIA in patients with CHD
Methodological detailssingle-centre
- Single-centre
Main finding
As reported in the abstract
- Surgical repair of CHD during first year of life
- 5
- Thymectomy at time of cardiac surgery
- 4
- Fever (after surgical intervention)
- 5
- Rash (after surgical intervention)
- 5
- Arthritis (after surgical intervention)
- 4
- Disease onset before 2 years of age
- 5
- Elevated IL-18 levels
- 5
- Baseline eosinophilia before bDMARD initiation
- 4
- HLA-DRB1*15:01 positivity
- 4
- Macrophage activation syndrome (MAS)
- 3
- sJIA-associated lung disease (sJIA-LD)
- 4
Observational data: associations, not effects.
Further findings
Death from complications of cardiac and/or pulmonary disease
- Deaths
- 2
Original publication
Severe Features of Systemic Juvenile Idiopathic Arthritis in Patients With Congenital Heart Disease. J Rheumatol. Published 1 June 2024. doi:10.
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