Physical Functioning, Pain, and Health-Related Quality of Life in Adults With Juvenile Idiopathic Arthritis: A Longitudinal 30-Year Followup Study.

OBJECTIVE: To describe physical functioning, pain, and health-related quality of life (HRQoL) in adults with juvenile idiopathic arthritis (JIA), investigate changes over time, and identify predictors of poorer HRQoL after 30 years of disease. METHODS: Patients (n = 176) clinically examined after 15 years were reassessed using the Health Assessment Questionnaire disability index (HAQ DI), the visual analog scale pain subscale (VAS pain), and the Medical Outcomes Study Short Form 36 (SF-36) after 23 years and 30 years. Patients with signs of active disease after a minimum of 15 years were clinically examined again at 30 years. Patients were compared to matched controls. RESULTS: At the 30-year followup, 82 patients (47%) had HAQ DI scores >0, and the median VAS pain score in patients was 0.6 (range 0-10). Patients had lower SF-36 physical component summary (PCS) scores compared with controls (P < 0.001), and this was evident for patients both with and without clinical remission (P ≤ 0.01). No group differences were found in SF-36 mental component summary scores. Patients also scored worse than controls on all SF-36 subscales (P ≤ 0.01) except mental health. PCS scores worsened significantly between the 15- and 30-year followup time points (P = 0.001). Worse HAQ DI, VAS pain, and patient's global assessment of well-being scores, and receiving disability/social living allowance at 30 years, were correlated with lower PCS scores. Worse HAQ DI, patient's global assessment of well-being, and VAS fatigue scores at 15-year followup predicted lower PCS scores at 30-year followup. CONCLUSION: JIA had a detrimental effect on physical HRQoL as measured by the PCS of the SF-36. The strongest correlates were physical disability, pain, fatigue, well-being, and receiving disability/social living allowance.

Radiographic damage in hands and wrists of patients with juvenile idiopathic arthritis after 29 years of disease duration.

BACKGROUND: There are few studies on radiographic outcome after long-term disease duration in juvenile idiopathic arthritis (JIA). We wanted to evaluate 29-year radiographic outcome in hands/wrists and predictors of damage in patients with long-term active JIA. METHODS: Patients diagnosed from 1980 to 1985, who had active disease at 15-, 23- or 29-year follow-up and arthritis in the wrists during the disease course, were reexamined with radiographs of hands/wrists. We used the adapted version of the Sharp van der Heijde (aSvdH) score and Carpal Height Ratio (CHR) to evaluate radiographic outcome. RESULTS: Sixty patients, mean age 38 years, were reexamined at median 29-year follow-up. 33 patients (55%) had an aSvdH score >0, median score was 4.0 (range 0-313), and 25% of the scores were high (≥53). Most patients with radiographic damage (88%) had both erosions and JSN. 52% of the patients had damage in the wrists, 43% in the MCP joints and 40% in the PIP joints. The CHR correlated strongly with the aSvdH. Both scores had high correlations with the Juvenile Arthritis Damage Index and the number of joints with limited range of motion (LROM) (rs = -0.688 to 0.743, p ≤ 0.001). The aSvdH correlated weakly with measures of disease activity. The number of joints with LROM, ESR and the HAQ disability score at 15 years and HLAB27 positivity predicted the aSvdH score and the CHR at 29-year follow-up. CONCLUSIONS: The majority of patients with long-term active JIA had modest radiographic damage, but more frequently in wrists than in fingers. The radiographic scores correlated well with measures of disease damage. Restricted mobility in joints at 15 years was the most important predictor of radiographic damage at 29 years.

Disease progression into adulthood and predictors of long-term active disease in juvenile idiopathic arthritis.

OBJECTIVES: To describe disease activity 30 years after disease onset in a previously studied cohort of patients with juvenile idiopathic arthritis (JIA) and reveal predictors of long-term active disease. METHODS: Patients with JIA, first referred 1980-1985 and re-examined 15 and 23 years after onset, were invited to attend. All 176 patients were assessed by questionnaires. Patients with signs of active disease at 15 years or later also came to a clinical re-examination (n=90). Disease activity was assessed by the clinical juvenile arthritis disease activity score (JADAS3) and by the criteria for remission in JIA, and health status by Health Assessment Questionnaire (HAQ) and Medical Outcome Study 36-item Short Form Health Survey (SF-36). RESULTS: At 30-year follow-up, 59% of the patients were in clinical remission off medication, 7% were in remission on medication and 34% had active disease. 70% of the patients were in the same category of disease activity at 15 and 30 years. The JADAS3 was ≤2.0 in 54%, 2.1-4.5 in 18% and >4.5 in 28%. HLA-DRB1*01, physician's global assessment and a short total time in remission at 15 years, predicted active disease. Physician's global assessment also predicted a JADAS3 >4.5. From 15 to 30 years (n=90), physician's global assessment, number of active joints, erythrocyte sedimentation rate and C reactive protein improved significantly, but patient's global assessment, HAQ and SF-36 did not. CONCLUSIONS: 41% of the patients with JIA had active disease or were on medication after 30 years and 28% had a high symptom state. Remission rates and patient-reported health status at 15 years were comparable with rates at 30 years.

Cardiac Function in Adult Patients with Juvenile Idiopathic Arthritis.

OBJECTIVE: To compare cardiac function in adults with longterm juvenile idiopathic arthritis (JIA) with that of healthy controls, and to investigate the influence of inflammation, disease severity, and use of antirheumatic medication on cardiac function. METHODS: Eighty-five patients with JIA (median age 38.6 yrs) with active disease for at least 15 years were reexamined at a median of 29 years after disease onset and compared with 46 matched controls. Echocardiography, including tissue Doppler imaging and longitudinal peak-systolic global strain, was used to assess diastolic and systolic myocardial function, and 12-channel electrocardiography was performed. RESULTS: The interventricular septum was thicker in patients than controls (mean ± SD 0.8 ± 0.2 cm vs 0.7 ± 0.1 cm, p = 0.036). Diastolic function in patients was altered compared with controls characterized by lower mitral E wave deceleration time (165 ± 36 ms vs 180 ± 40 ms, p = 0.029), higher surrogate marker of left ventricular (LV) filling pressure (median lateral E/e' 5.3, interquartile range 4.6-6.3 vs 4.8, 3.9-5.7, p = 0.036), and larger left atrial area (16.4 ± 2.9 cm(2) vs 15.1 ± 2.8 cm(2), p = 0.015). Systolic and diastolic blood pressures were higher in patients (120 ± 15 mmHg vs 114 ± 9 mmHg, p = 0.021 and 76 ± 10 mmHg vs 71 ± 8 mmHg, p = 0.009, respectively). QT corrected interval was similar in patients and controls. High high-sensitivity C-reactive protein (CRP), polyarticular disease course, and extended joint affection at 29-year followup, as well as duration of active disease, cumulative erythrocyte sedimentation rate, and CRP, and prednisolone use were associated with higher lateral E/e'. CONCLUSION: Adult patients with JIA did not differ from controls in LV systolic function, but had mildly thicker interventricular septum and indications for higher LV filling pressure, and most in patients with a higher disease burden.

Arterial haemodynamics and coronary artery calcification in adult patients with juvenile idiopathic arthritis.

OBJECTIVE: To compare arterial haemodynamics in adults with long-term juvenile idiopathic arthritis (JIA) to that of healthy controls, and explore the influence of traditional cardiovascular risk factors and disease characteristics on arterial haemodynamics plus coronary artery calcification. METHODS: 87 JIA patients (median age 38.4 years) with persistently active disease at least 15 years after disease onset (registered by longitudinal follow-up), were re-examined after median 29 years and compared with 87 matched controls. Arterial haemodynamics were characterised by arterial stiffness and blood pressure. Sphygmocor was used to measure the arterial stiffness markers pulse wave velocity (PWV) and augmentation index (AIx). Coronary calcification was assessed by CT. RESULTS: Compared to controls, patients had significantly higher PWV (7.2 vs 6.9 m/s, p=0.035), and systolic and diastolic blood pressure (SBP, p=0.050 and DBP, p=0.029). AIx was numerically higher in the patients compared to the controls, but no statistically significant difference was found. Coronary calcification was present in 22 (26%) of the patients. Daily smoking was more frequent (p=0.043), and insulin resistance was higher (p=0.034) in patients than controls.In patients, DBP, but no disease variables were determinants of PWV. Disease variables as well as traditional cardiovascular risk factors were associated with higher AIx, DBP and the presence of coronary calcification. CONCLUSIONS: JIA patients with long-term active disease had altered arterial haemodynamics compared with controls in our study. PWV was mainly determined by increased DBP, a parameter that again was associated with JIA disease and treatment variables.