The early clinical recognition of juvenile-onset ankylosing spondylitis and its differentiation from juvenile rheumatoid arthritis

Abstract

Objective: To determine which early clinical data differentiate juvenile-onset ankylosing spondylitis (AS) from juvenile rheumatoid arthritis (JRA).

Methods: Medical records of 35 patients with juvenile-onset AS and 75 with JRA (excluding type II pauciarticular JRA), all of whom had disease onset at age < or = 16 years, disease duration of < or = 2 1/2 years at the initial visit to the rheumatology clinic, and followup of > or = 10 years, were analyzed retrospectively with regard to features of disease found 6 months, 12 months, and 10 years after onset.

Results: At 6 months, various features appeared more frequently in the juvenile-onset AS group than in the JRA group, i.e., pauciarthritis (54.3% versus 30.7%; P = 0.03, odds ratio [OR] = 2.7), enthesopathy (82.9% versus 0%; P < 0.0001, OR = 321.4), tarsal disease (71.4% versus 1.3%; P < 0.0001, OR = 185.0), and lumbar/sacroiliac symptoms (11.4% versus 0%; P = 0.02, OR = 11.9). At 12 months, the features found more frequently among juvenile-onset AS patients than JRA patients were enthesopathy (88.6% versus 4.0%; P < 0.0001, OR = 186.0), tarsal disease (85.7% versus 10.7%; P < 0.0001, OR = 50.3), and knee disease (100.0% versus 82.7%; P = 0.04, OR = 8.0). Involvement of the upper extremities (especially the hands) was found in significantly fewer juvenile-onset AS patients compared with the JRA group. Definite involvement of the spine and sacroiliitis in juvenile-onset AS occurred after a mean +/- SD of 7.3 +/- 2.0 years.

Conclusion: Regardless of axial disease, enthesopathy and tarsal disease in children who have arthritis of the lower, but not of the upper extremities differentiate juvenile-onset AS from JRA within 1 year of symptoms. The discriminative value of these parameters approaches that of axial disease (the gold standard) throughout the followup period.