Abstract
Introduction: Systemic onset Juvenile idiopathic arthritis (sJIA), differs from other JIA subtypes in its clinical manifestations and pathogenesis. It is characterized by a severe course and has the highest rate of mortality and morbidities. Objectives: To study initial manifestations, laboratory findings, course, outcome and complications of patients of sJIA in pediatric rheumatology clinic in Tripoli Children Hospital, Which is one of two referral clinic covering the western and southern part of Libya Methods: The files of the patients diagnosed with sJIA from 5/2000 t0 5/2021 were reviewed and data was retrospectively collected regarding the presenting symptoms, initial investigations, disease course, complications and treatment used. Patients who followed up for less than 6 months were excluded. Results: There were 38 patients with sJIA diagnosed according to the International League Against Rheumatism (ILAR) criteria were included. 47.4% of them were males and 52.6% were females. They were followed up for a period of 6.3±4.4y. The mean period from appearance of symptoms to first follow up in rheumatology clinic 5±6 months. The following table summarizes the most common presenting symptoms among the patients: There is one patient presented with MAS and required ICU management. The most frequently affected joints are the knees (76.3%) followed by the ankles (73.7%) then the wrists (55.3%) and the elbows (39.5%). The disease course was monocyclic in 39.5%, polycyclic in 28.9% and persistent in 31.6% of the patients. and in 65.8% the disease has polyarticular pattern while in the rest of the patients it followed an oligoarticular pattern. The number of patients who achieved remission is 30 (78.9%), 16 (53.3%) of them were in remission on treatment while 14 (46.7%) of them were in remission off treatment Oral steroids, NSAIDs and MTX were used as a first line treatment to control the disease in the patients. Also intraarticular injections were used in 21.1% of the patients. In 55.3% of the patients switching to biologics was necessary to control the disease, Etanercept was sufficient in controlling the disease in only 2 patients (5.2%) while Anakinira was sufficient in controlling the disease in 5 patients (13.2%) and Tocilizumab was sufficient in controlling the disease in 6 patients (15.8%). More than one biologic used in 8 patients. Joint deformities were present in 10 (26.3%) of the patients, and evidence of extra-articular damage is present in 12 (31.6%). MAS occured in 4 (10%) of the patients and one patient experienced 3 mas episodes Conclusion: There is a high rate of disease remission on and off treatment and high rate of disease related articular and extraarticular damage because alot of patients started biologics late after disease onset. Patient Consent Received No