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Arthropathy of Down's syndrome
Arthritis and Rheumatism
|August 1, 1984
Summary
Children with Down's syndrome (trisomy 21) can develop arthritis, often responding to NSAIDs but rarely achieving remission. This condition warrants exclusion from juvenile rheumatoid arthritis diagnoses.
Area of Science:
- Rheumatology
- Genetics
- Immunology
Background:
- Down's syndrome (trisomy 21) is linked to skeletal issues, joint hypermobility, and immune system abnormalities.
- An increased incidence of autoimmune phenomena is observed in individuals with Down's syndrome.
Observation:
- This study reports on 7 pediatric patients diagnosed with Down's syndrome and arthritis.
- The arthritis presented as polyarticular in four children and pauciarticular in three at onset.
- Cardiac disease was significant in only one patient; HLA typing showed no specific correlations.
Findings:
- All patients responded to nonsteroidal antiinflammatory drugs (NSAIDs), with only one achieving clinical remission.
- Two patients died during the follow-up period: one from cervical spine instability and another from cardiac compromise.
- Arthropathy in Down's syndrome should be considered an exclusion criterion for juvenile rheumatoid arthritis.
Implications:
- Investigating the link between Down's syndrome and arthritis may elucidate the interplay of genetic and immunologic factors in joint inflammation.
- Recognizing this distinct arthropathy is crucial for accurate diagnosis and management in children with Down's syndrome.