Oral (15A119)

Down’s Arthropathy (DA): Clinical and Radiological Features of Arthritis in Children with Trisomy 21 (T21)

Author(s)

C Foley, EJ MacDermott, D Veale, OG Killeen

Department(s)/Institutions

NCPR OLCHC; SVUH

Introduction

The ‘Arthropathy of Down syndrome’ was first described in 1984, yet we still have limited literature on the features of this arthritis, although it is estimated to be 3-6 times more common than JIA in the general paediatric population.

Aims/Background

To describe the clinical course and radiological features of DA.

Method

Musculoskeletal examination performed on children with T21 (0-20 years). DA cases identified, investigated and managed as per normal clinical practice. New cases of JIA diagnosed, recruited to a comparison group.

Results

503 children with T21 screened for arthritis (56.4% Male, age 0.6-19.2 years); 22 new cases of DA detected (33 = total DA cases now attending the NCPR).

DA Features
• 82% = Polyarticular RF negative arthritis,
• 78.6% = Small joint involvement,
• 66.7% = X-Ray changes at diagnosis, (29.2% erosions),
• 1.7 years = Time to diagnosis
• 75% = Methotrexate (MTX)- associated nausea.

Conclusions

Small joint involvement of the hands is a predominant disease pattern, and unique to DA. Treatment with MTX was complicated by significant MTX-associated nausea. Outcomes could be improved for children with DA by inclusion of a musculoskeletal examination in the annual T21 surveillance programme.

References

Yancey, CL. Zmijewski, C. Athreya, BH and Doughty, RA. (1984)

Arthropathy of Downs syndrome. Arthritis Rheum 27(8): 929-34 Olson, JC. Bender, JC. Levinson, JE. Oestreich, A and Lovell, DJ. (1990)

Arthropathy of Down syndrome. Pediatrics 86(6):931-6.