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S U M M A R Y
Dept. Of Pediatric Rheumatology, University Child Clinic, UCC Nis
ETIO-PATOGENTIC DILEMAS IN SYSTEMIC JUVENILE IDIOPHATIC ARTHRITIS
JELENA VOJINOVIC
Diagnosis of SJIA is dificult to be establish especialy to periodic fever syndroms (HIDS, TRAPS, PFAPA etc.).
Clinical presentation of the disease as well as immunoregulatory specificity makes difference compared to other
forms of JIA. Apearance of macrophage activation syndrome (MAS), as a sever complication of this specific JIA
type, provoke doubt should it be concidered auto-inflammatory instead auto-immunity sindrom. Geneticaly it is
copmlex disease conected to HLA genes in 15-17% of patients. Genes coding other immunoregulatory molecules,
like MIF gen, IL-6 gene polimorphism, MEFV gen etc., play importante role in disease pathogenesis. Increase of
CCR5+ and CXCR3+ T-lymphocytes, as markers of predominant Th1 immunoregulation, deficient production of
perforine and granzyme B and deficient apoptosis ponts out importance of T cell mediated immunoregulation in
SJIA. Increase of CCR5+ and CXCR3+ T-lymphocytes, as markers of predominant Th1 immunoregulation,
deficient production of perforine and granzyme B and deficient apoptosis ponts out importance of T cell mediated
immunoregulation in SJIA. Changed immunoregulatory functions, citokine production, latent infection and
immunodeficiency, together play major etio-pathogenetic mechanism.
Key woords:Systemic onset juvenile idiopathic arthritis, autoimmunity, etiopathogenesis