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Vol. 24, Art. 15 (pp. 176-199)    |    2023       

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Systemic juvenile idiopathic arthritis and its biomarkers (literature review)

Khalchitsky S.E.1, GrachevaYu. A.1, Pechal`nova S.A.1, Buslov K.G.1, , Vissarionov S.V.1, Batotsyrenova E.G.2, Komov Yu.V.2, Kretser T.Yu.2, Kashuro V.A.2,3,4

1H.Turner National Medical Research Center for Children's Orthopedics and Trauma Surgery
2 St. Petersburg State Pediatric Medical University, St. Petersburg, Russia
3Herzen State Pedagogical University of Russia, Saint Petersburg, Russia
4Saint Petersburg State University, Saint Petersburg, Russia

Brief summary

Systemic juvenile idiopathic arthritis (sJIA) and its complication, macrophage activation syndrome (sJIA-MAS), are rare but sometimes very serious or even critical diseases of childhood that can sometimes be characterized by non-specific clinical signs and symptoms at onset, such as persistent fever, headache, rash or arthralgia - accompanied by elevated levels of acute phase reagents. For a correct affirmative diagnosis, bacterial or viral infections, neoplasms, and other immune-mediated inflammatory diseases must be excluded. Delay in diagnosis results in late initiation of targeted therapy. A set of biomarkers is useful in distinguishing sJIA or sJIA-MAS from similar clinical conditions, especially in the absence of arthritis. Biomarker analysis should be accessible to patients, have acceptable production and acquisition costs for medical laboratories, and also be fairly easy to determine, have high sensitivity and specificity, and correlate with pathophysiological pathways of the disease. At present, there is a need to identify the most significant biomarkers and their synergistic interaction for simple and accurate recognition of sJIA and sJIA-MAS in order to immediately guide clinicians in the direction of correct diagnosis, predicting disease outcomes, response to treatment, and risk of relapse. sJIA biomarkers represent an exciting area of research at present, especially due to the heterogeneous nature of cytokine storm syndromes (CSS) in the COVID era. They must be chosen with great care - a fact supported by the increasingly improved genetic and pathophysiological understanding of sJIA as well as CSS - so that new classification systems can soon be developed to correctly differentiate similar groups of patients with diseases of different origins. The review provides information on the latest advances in the development and use of biomarkers in systemic juvenile idiopathic arthritis. PubMed articles were searched combining predefined keywords related to systemic JIA biomarkers, its complications, and comorbidities.


Key words

juvenile arthritis, macrophage activation syndrome, biomarkers, cytokines, chemokines, predictors, remission, relapse, treatment response

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