MANIFESTATIONS PECULIARITIES OF JUVENILE ARTHRITIS WITH SYSTEMIC ONSET IN PATIENTS WITH THE RESPIRATORY LESIONS’ DEVELOPMENT AT THE ONSET OF THE DISEASE. RESULTS OF A SINGLE-CENTER RETROSPECTIVE COHORT STUDY AND A BIBLIOGRAPHICAL REVIEW

Abstract

Juvenile arthritis with systemic onset (sJIA) is one of the most difficult tasks of differential diagnosis and selection of therapy for a rheumatologic practitioner as yet. The presence of a systemic hyperinflammatory response, the development of macrophage activation syndrome (MAS) and sepsis determine the severity of sJIA. Pulmonary involvement coupled with the development of interstitial lung disease and/or pulmonary arterial hypertension is a relatively new, potentially life-threatening complication of sJIA. The purpose of this research was to characterize patients with sJIA who had respiratory lesions (RL) at the onset of the disease. Methods used: a retrospective cohort study included the data from the case histories of 85 pediatric patients with sJIA who were divided into 2 groups: G1 with RL (dyspnea, pleurisy, interstitial lung disease, acute respiratory distress syndrome); and G2 of those who’ve had no such complaints. Results: 18 (21.1%) had RL associated with female gender, heart lesions (OR=6.4 (2.1; 19.7), p=0.0006), macrophage activation syndrome (72.2%; OR=6.6 (2.1; 21.0), p=0.0006), hemorrhagic syndrome, CNS disorders (OR=6.1 (1.2; 30.3), p=0.015), decreased levels of hemoglobin, platelets, prothrombin, fibrinogen, albumin, total protein and sodium as well as the higher levels of ferritin, lactate dehydrogenase, aspartate aminotransferase, triglycerides. Patients with lung involvement had tended to have higher C-reactive protein levels and were more likely to have transient proteinuria in the active phase of the disease. Multivariate analysis identified two criteria associated with the respiratory damage: heart damage (β=0.18, p=0.039) and hypoalbuminemia <26 g/l (β=0.73, p=0.0000001). Conclusion: the presence of macrophage activation syndrome, damage to the cardiovascular system requires targeted monitoring of respiratory damage in patients with sJIA.