Background In 1999 a registry of patients with juvenile idiopathic arthritis and juvenile onset connective tissue diseases was established at Oslo University Hospital, Rikshospitalet.
Objectives To evaluate clinical characteristics in patients with juvenile onset connective tissue diseases registered in the hospital based registry.
Methods The registry is based on written informed consent. Patients with juvenile onset connective tissue diseases were classified using ICD-10 codes and data was registered at one time point. Gender, date of registration, disease onset, Childhood Health Assessment Questionnaire (CHAQ) and self-reported assessment of pain and wellbeing (VAS 0-10cm, where 0 indicates no pain and excellent wellbeing) was recorded, in addition to disease-specific variables. CHAQ score (range 0-3) was calculated without corrections for helping aids. For patients under the age of 16 years, both VAS and CHAQ were reported by the parent. Data is shown as n (%) or median (IQR).
Results A total of 137 patients with juvenile onset connective tissue diseases were registered. Median age at disease onset was 12 years and median age at inclusion was 14 years, and 77% were female. The distributions in various ICD-10 subgroups are shown in table 1. The most frequent subgroups were systemic lupus erythematosus (SLE), juvenile dermatomyositis (JDM), mixed connective tissue disease (MCTD) and Wegener's granulamatosis. Assessment of wellbeing was worse in girls (n=89) than in boys (n=26) in the group as whole; median 23 (10-49) versus median 7 (1-41); p=0.050. When comparing levels of wellbeing between different diagnoses, no differences were found. Pain intensity was higher in patients with MCTD than in those with Wegener's (p=0.016). No difference was found when comparing assessment of pain between genders. Median CHAQ score was 0.0 for all diagnoses, except for JDM and MCTD (0.6, 0.3, respectively). A higher CHAQ score was found in patients with JDM compared to SLE (p=0.001) and Wegener's patients (p=0.006), and in patients with MCTD compared to SLE patients (p=0.05).
Conclusions The age at onset and gender distribution is as expected. MCTD patients seemed to have a worse experience of pain than the other patients. Girls had lower sense of wellbeing than boys. Limited physical functioning was more frequent in patients with JDM and MCTD than in other patient groups. The registry is an important database for further studies concerning juvenile onset connective tissue diseases.
Disclosure of Interest None declared