Systemic Juvenile Idiopathic Arthritis and Secondary Macrophage Activation Syndrome in Latvia from 2009 to 2020: A Nationwide Retrospective Study

Kristīne Lukjanoviča (Coresponding Author), Ieva Šlēziņa, Zane Dāvidsone, Ruta Šantere, Kristīna Budarina, Valda Staņēviča

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Article number798
JournalMedicina (Kaunas, Lithuania)
Issue number4
Publication statusPublished - 20 Apr 2023


  • juvenile arthritis
  • systemic juvenile idiopathic arthritis
  • sJIA
  • still’s disease
  • macrophage activation syndrome;
  • MAS
  • tocilizumab

Field of Science*

  • 3.2 Clinical medicine

Publication Type*

  • 1.3. Anonymously reviewed scientific article published in a journal with an international editorial board and is available in another indexed database


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