According to modern science, systemic juvenile arthritis (sJA) is an autoinflammatory disease characterized by severe systemic manifestations and life-threatening complications. This article discusses the developmental predictors, clinical and radiological manifestations and pathogenetic features of the disease. Particular attention is paid to genetically engineered biological therapy. Numerous pulmonary complications are now known in sJA children, including interstitial lung disease (ILD), pulmonary alveolar proteinosis (PAP), pulmonary hypertension (PH), and lipoid pneumonia. Fatality rate in sJA patients increases against the macrophage activation syndrome (MAS) development and pulmonary hypertension, which occurs with proliferation of endothelial cells, muscle tissue and fibroblasts in the lungs vessels. A more severe disease progression is typical for children with genetic defects. SAM and PAP relapses are observed more often in such patients. Instrumental diagnostic methods helped to identify, 5 subtypes determining the lung tissue damage in sJA. Computer tomography (CT) revealed the main signs of lung damage in sJA patients: ground-glass opacity, crazy-paving sign, thickening of the bronchial wall, interlobar septum, pleura, peripheral consolidation, and lymphadenopathy. Due to the high level of sJA activity, children were prescribed genetically engineered biological drugs (GEBP). Timely therapeutic correction is necessary to exclude life-threatening adverse reactions. Under dynamic observation, it is possible to diagnose lung damage in children at the early stage and to control the pathology. The purpose of this review is to systematize the existing data on developmental predictors, pathogenetic features of the disease, sJA clinical and radiological manifestations, and genetically engineered biological therapy as a method of sJA treatment.