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Juvenile arthritis in Turner's syndrome: a multicenter study
F Zulian1, H R Schumacher, A Calore
1Department of Pediatrics, University of Padova, Italy.
Clinical and Experimental Rheumatology
|August 26, 1998
Summary
Juvenile arthritis is a newly identified autoimmune condition associated with Turner's syndrome (TS). This study found a significantly higher prevalence of juvenile arthritis in individuals with TS than expected by chance.
Area of Science:
- Pediatric Rheumatology
- Genetics
- Autoimmunology
Background:
- Turner's syndrome (TS) is a chromosomal disorder linked to various autoimmune conditions.
- Previous research has documented associations between TS and thyroiditis, inflammatory bowel disease, and diabetes.
Purpose of the Study:
- To investigate the association between Turner's syndrome and juvenile arthritis (JA).
- To determine the prevalence and characteristics of JA in patients with TS.
Main Methods:
- A survey was conducted across 28 pediatric rheumatology centers in the USA, Europe, and Canada.
- Data on patients with both TS and juvenile arthritis were collected and analyzed.
Main Results:
- Eighteen cases of TS were identified among approximately 15,000 juvenile rheumatoid arthritis (JRA) patients.
- Two patterns of arthritis were observed: polyarticular (early onset, seronegative, deforming) and oligoarticular (benign, ANA+).
- Synovial tissue analysis revealed chronic inflammation and hyperplasia, consistent with other forms of JRA.
Conclusions:
- Juvenile arthritis represents a novel autoimmune association with Turner's syndrome.
- The prevalence of JA in TS patients is at least six times higher than random association.
- Synovial findings in TS-associated JA are indistinguishable from other JRA forms.