Background/Purpose:

The aim of the study was to assess disease activity and health status in a previously studied cohort of patients with juvenile idiopathic arthritis (JIA) 30 years after disease onset and reveal predictors of active disease. 

Methods:

A total of 254 patients with JIA, first referred to our hospital from 1980 to1985, were reexamined clinically after median 15 years of disease duration, and by mailed questionnaires after median 23 years. These patients were invited to attend the present study. All patients were assessed by questionnaires, and those with signs of active disease after 15, 23 and/or 30 years were invited to a clinical reexamination. Health status was measured by HAQ and SF-36, and disease activity by the clinical version of the juvenile arthritis disease activity score (cJADAS). Logistic regression analyses were used to assess predictors of persistently active disease and an unacceptable symptom state (cJADAS > 4.5).

Results:

One hundred and seventy-one patients (67%) were included in the study. They were examined after a median of 30 (range 21-40) years of disease duration, median age 39 (range 28-45) years, 74% females.

              After 30 years, 101 patients (59%) were in clinical remission off medication, 12 (7%) were in remission on medication and 58 (34%) had persistently active disease. Thirty-seven of 57 patients (65%) with active disease at 15 years follow-up had active disease at 30 years follow-up, and 20 patients (35%) went into remission on/off medication. Eighty-four of 97 patients (87%) in remission off medication at 15 years were in remission at 30 years follow-up. Patients in remission on medication at 15 years (n=17) tended to flare (n=9) or go into remission off medication (n=6).

The cJADAS score was median 1.9 for the total study group, and 43% (n=73) of the patients had a cJADAS score ≤ 1.0, 10 % (n=16) had cJADAS from 1.1 to 2.0, 18% (n=30) had cJADAS from 2.1 to 4.5, and 30% (n= 50) had cJADAS > 4.5.

Predictors of persistently active disease at 30 years follow-up were: being diagnosed with a JIA subgroup other than persistent oligo articular and systemic JIA (OR 4.1, 95%CI 1.5-11.5), being DR1 positive (OR 8.3, 95%CI 2.3-30.3), a short total time in remission (OR 9.0, 95% CI 3.0-26.7), and not being in remission at 15 years follow-up (OR 13.7, 95%CI 4.9-38.4). R²=65%. Predictors of a cJADAS score > 4.5 at 30 years follow-up were physician’s global score of disease activity at 15 years (OR 2.4, 95%CI 1.7-3.5) and daily smoking (OR 2.5, 95%CI 1.1-5.9). R²=23%.

We compared disease activity at 15 years with that of 30 years follow-up for 90 patients. There were significant improvements in physicians global assessment of disease activity (p=0.003), number of active joints (p=0.010), ESR (p=0.041) and CRP (p<0.001), but no significant change in patient’s global assessment, number of joints with limitation of motion, HAQ, or SF-36.

Conclusion: The overall remission rates were stable between 15 and 30 years. After 30 years, a third of the JIA patients had an unacceptable symptom state and a third was not in remission on or off medication. Physician’s assessment of disease activity and inflammatory markers improved over the years, but patient reported disability and health status did not change.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/disease-progression-into-adulthood-in-patients-with-juvenile-idiopathic-arthritis-a-longitudinal-30-year-follow-up-study/