Systemic onset juvenile idiopathic arthritis a single-center experience at Tripoli Children's Hospital, Libya

Systemic onset Juvenile Idiopathic Arthritis (sJIA) is a severe subtype of juvenile idiopathic arthritis, characterized by significant morbidity and mortality due to its multisystem involvement and risk of life-threatening complications such as macrophage activation syndrome. This study aimed to characterize the demographic and clinical profile of sJIA patients managed at the Tripoli Children's Hospital. Medical records of pediatric patients diagnosed with sJIA according to ILAR criteria, those who received follow-up care during 2021 were reviewed. Of 35 records, 32 patients met the inclusion criteria. Findings indicated that female patient's prevalence by 71.9%. The mean age at diagnosis was 6.28 years, with male patients diagnosed significantly younger than female. Referral times varied with 25.0% referred within two months, 46.9% between 2-6 months, and 28.1% experiencing delays exceeding six months. No association was found between gender or age at diagnosis and referral time. Clinical presentation often included a triad of fever, arthritis, and rash. Skin rash was observed in 65.6% of the patients, lymphadenopathy in 25.0%, of the hepatosplenomegaly in 9.0% of patients, and serositis in 6.3% of patient. Large joints were most frequently involved, with the knee and wrist being the most common. Oligoarticular pattern was present in 75.0% of the patients, while 25.0% had polyarticular pattern. Joint deformities were observed in 40.0%, with the knee being the most affected. Other complications included short stature, cataract, osteoporosis, and delayed puberty. Macrophage activation syndrome developed in certain patients, mostly in female patients. Delayed referral (exceeding six months) was associated with more extensive joint involvement and a broader spectrum of complications, with 33.3% showing no disease damage, compared to of those referred within two months. This study highlights the demographic and clinical characteristics of sJIA in Libya, emphasizing the significant burden of complications and the impact of delayed referral on disease outcomes.