Analysis of disease activity, functional disability and articular damage in patients with juvenile idiopathic arthritis: a prospective outcome study.

OBJECTIVES: We longitudinally studied outcomes of patients with juvenile idiopathic arthritis (JIA) using the Childhood Health Assessment Questionnaire (CHAQ) for physical disability and the Juvenile Arthritis Damage Index for articular (JADI-A) and extra-articular damage (JADI-E), and we correlated them with various disease activity variables. METHODS: Eighty-seven patients with JIA were included in the prospective follow-up study with median age 14 years (4.6-18.0), disease duration 5.2 years (2.0-18.9) and follow-up of 4.0 years (2.0-5.2). Besides JADI-A and JADI-E, and the assessment of active joints count, joints with limited mobility, ESR, CHAQ and radiographic damage of joints was also done. A correlation analysis of CHAQ and JADI with various disease activity variables was performed. RESULTS: The patient's distribution of JIA subtypes were polyarticular (32), systemic onset (13), oligoarticular (31), and enthesitis related arthritis (11). After a follow-up period, 46% patients had active disease compared to 83% patients at baseline (p<0.01). The CHAQ disability index improved over baseline, while radiological damage (p<0.001) and JADI-A and JADI-E scores worsened (p<0.001). CHAQ and JADI significantly correlated with the majority of disease activity variables. CHAQ DI was significantly higher in the patients with coxitis (p<0.01) and wrist arthritis (p<0.001). The most pronounced deterioration in articular damage (JADI-A) was observed in patients with sJIA (3.69 at baseline vs. 5.69 at study endpoint). CONCLUSIONS: The improvement of functional disability (CHAQ DI) was observed over the course of the disease, whereas radiological joint damage, JADI-A and JADI-E scores worsened. Children with systemic JIA, wrist arthritis, coxitis and prolonged active disease are at higher risk of progression of severe disability.

Major salivary gland sonography in Sjögren's syndrome: diagnostic value of a novel ultrasonography score (0-12) for parenchymal inhomogeneity.

OBJECTIVE: To validate ultrasonographic criteria for examination of the major salivary glands in the diagnosis of primary Sjögren's syndrome (SS). METHOD: A total of 209 consecutive patients with rheumatic diseases were selected according to the American-European Consensus Group (AECG) classification criteria for SS. One hundred and fifteen patients had primary SS, 44 had secondary SS, and 50 had sicca symptoms, and 36 subjects served as asymptomatic controls. This cohort was analysed for size, echogenicity, parenchymal inhomogeneity, focal changes, and posterior borders of the major salivary glands by ultrasonography (US). A novel US score for parenchymal inhomogeneity (0-12) was assigned and its diagnostic accuracy evaluated. RESULTS: Ultrasonographic abnormalities of salivary glands were detected in 107/115 (93.0%) patients with primary SS, in 12/44 (27.3%) with secondary SS, in 25/50 (50.0%) with sicca symptoms, and in 4/36 (11.1%) asymptomatic controls. Area under the receiver operating characteristic curve (AUC-ROC) for US inhomogeneity score was highly significant [0.96 +/- 0.01; 95% confidence interval (CI) 0.94-0.99, p < 0.000] for primary SS, with a sensitivity to specificity ratio of 91/83 for parotid and 93/90 for submandibular glands. Setting the cut-off US inhomogeneity score at 6 resulted in the best ratio of specificity (90.0%) to sensitivity (95.1%), with a positive predictive value of 72% and a negative predictive value of 96%. A US inhomogeneity score >or= 6 was closely correlated with positive biopsy (p < 0.000) and scintigraphy findings (p < 0.000). CONCLUSIONS: We demonstrate the high diagnostic value of a novel US score for parenchymal inhomogeneity (0-12) that could serve as a useful single US criterion in the evaluation of salivary gland involvement in primary SS.