Clinical, paraclinical and evolutionary features of juvenile idiopathic arthritis, systemic form

Abstract

Background. Systemic juvenile idiopathic arthritis (SJIA) is an inflammatory disease that can cause fever, arthritis, and sometimes rash. It can also cause extensive lymphadenopathy, hepatosplenomegaly, and serositis. The prevalence of SJIA ranges from 0 to 8.6/100 000, with incidence maxima occurring in children ages 1 to 5 years with both men and women equally. Objective of the study. To evaluate the clinical, paraclinical and evolutionary features of juvenile idiopathic arthritis, systemic form. Material and methods. The bibliographic sources for this study were analyzed using PubMed, Google Scholar, MedScape, Oxford Academic and were released between 2013-2023. Results. The typical laboratory findings of granulocyte predominant leukocytosis, elevated acute-phase reactants including thrombocytosis and hyper-ferritinemia, C-reactive protein (CRP) and very high ESR levels. The presence of intermittent, daily, high, spiking fevers (in a quotidian fever pattern), typical evanescent rash, and arthritis are used to make the clinical diagnosis. Additionally, alarmin proteins S100A8/A9 (calprotectin or MRP8/14) and S100A12 (calgranulin C), which are significantly raised in SJIA, are demonstrated to be significantly higher in serum markers of innate immune activation. Macrophage activation syndrome (MAS), a consequence of SJIA, should be immediately suspected in cases of mild increases in AST, ALT, hypoalbuminemia, elevated globulin level, and low-grade D-dimer positive. Imaging tests such as X-rays are rarely useful in the diagnosis of juvenile arthritis; however, an MRI or, on occasion, an ultrasound, can be used to screen for problems or to identify early joint inflammation. Inhibitors of IL-1 and IL-6 have been proven to be quite successful in treating SJIA, as these two factors are important in the disease’s pathophysiology. A possible “window of opportunity” in the treatment of children with this rare illness appears to be represented by recent results suggesting that early cytokine blockade may abrogate chronic, destructive, therapy-resistant arthritic phase. Conclusion. Systemic Juvenile Idiopathic Arthritis (SJIA) presents several challenges because of its complexity and variability. Specialized care is necessary for patients with erosive polyarthritis, long-term systemic disease, those who are not responding to standard therapies, and those who have remitting-relapsing Macrophage Activation Syndrome (MAS). To effectively manage a health issue, monitor its progression, and address its long-term effects to improve patient outcomes and lower morbidity and mortality, a multidisciplinary approach is essential.