Lukjanoviča, KristīneŠlēziņa, IevaDāvidsone, ZaneŠantere, RutaBudarina, KristīnaStaņēviča, Valda2023-05-032023-05-032023-04-20Lukjanoviča, K, Šlēziņa, I, Dāvidsone, Z, Šantere, R, Budarina, K & Staņēviča, V 2023, 'Systemic Juvenile Idiopathic Arthritis and Secondary Macrophage Activation Syndrome in Latvia from 2009 to 2020 : A Nationwide Retrospective Study', Medicina (Kaunas, Lithuania), vol. 59, no. 4, 798. https://doi.org/10.3390/medicina590407981010-660XPubMedCentral: PMC10144400https://dspace.rsu.lv/jspui/handle/123456789/11864Zane Davidsone is a representative of European Reference Network on Connective Tissue and Musculoskeletal Disease-ERN-ReCONNET.Background and Objectives: Systemic juvenile idiopathic arthritis (sJIA) is a distinctive JIA subtype with mostly nonspecific systemic clinical features, which can be a diagnostic challenge. This study aimed to analyze our experience with sJIA in Latvia for twelve years: assessing clinical and epidemiological characteristics, the efficacy of therapy, and disease outcomes, including the development of macrophage activation syndrome (MAS). Materials and methods: This is a descriptive study in which we conducted a retrospective case review of all patients with sJIA diagnosis admitted to the only pediatric tertiary centre in Latvia during the period 2009-2020. Results: sJIA was diagnosed in 35 patients with a mean annual incidence rate of 0.85 patients per 100,000 children. Major clinical signs at the first visit were: fever, rash, arthritis, and lymphadenopathy. Almost half of the patients, 48.5%, had a monocyclic disease course, and only 20% of patients had persistent disease. MAS developed in 28.6% of patients. Biological therapy was administered to 48.6% of patients, mostly by tocilizumab, which induced remission in 75% after one year, and in 81.2% after two years without any serious therapy-related complications. In our study, none of the patients had interstitial lung disease, drug reaction with eosinophilia and systemic symptoms (DRESS)-like syndrome, or fatal disease. Conclusions: The incidence and clinical characteristics of sJIA correlate with the literature findings, although MAS was more common than described in other studies. There is a tendency for the persistent disease to decrease with the use of biological therapy. Tocilizumab is an efficient choice of treatment with a good safety profile.309191enginfo:eu-repo/semantics/openAccessjuvenile arthritissystemic juvenile idiopathic arthritissJIAstill’s diseasemacrophage activation syndrome;MAStocilizumab3.2 Clinical medicine1.1. Scientific article indexed in Web of Science and/or Scopus database/dk/atira/pure/researchoutput/researchoutputtypes/contributiontojournal/article10.3390/medicina59040798http://www.scopus.com/inward/record.url?scp=85156178116&partnerID=8YFLogxK