Clinical characteristics and prognosis of young children with idiopathic arthritis combined with lung injury / 中华实用儿科临床杂志

ABSTRACT

Objective:To explore clinical manifestations, imaging features and prognosis of juvenile idiopathic arthritis (JIA) combined with lung injury, aiming to improve the understanding of the disease.Methods:Clinical data from 464 children with JIA who were hospitalized in Beijing Children′s Hospital from January 2016 to September 2019 were retrospectively analyzed.Their clinical manifestations, high resolution CT (HRCT) features, lung function and follow-up of children with lung injury were analyzed.Results:Among 464 children with JIA, 40 cases (8.62%) combined with lung injury.There were no significant differences in the age and sex between JIA children either combined with lung injury or not (all P>0.05). Among them, there were 125 cases of systemic-onset juvenile idiopathic arthritis (SoJIA) and 28 cases (22.4%) of JIA combined with lung injury, accounting for the highest proportion (70%, 28/40 cases) in JIA children combined with lung injury.Among 40 JIA children combined with lung injury, 22 cases (55.0%) had respiratory symptoms and 7 cases (17.5%) had obvious hypoxia.HRCT examination was performed in them, and the imaging findings included high-density strip or strip flocculation (75.0%, 30/40 cases), pleural thickening (45.0%, 18/40 cases), ground glass shadow (22.5%, 9/40 cases), nodular lesion (20.0%, 8/40 cases), vesicles or cystic emphysema (15.0%, 6/40 cases). Lung function was detected in 12/20 children with varying degrees of pulmonary function abnormalities, most of which were mixed ventilation dysfunction, and 2 cases still had pulmonary function abnormalities after treatment.During the follow-up for 3 months to 3.5 years, 4 cases (10%) JIA combined with lung injury died.A total of 29 children were re-examined by pulmonary CT in the follow-up visit, including 14 (48.28%) improved, 8 cases (27.58%) with no significant improvement, and 7 cases (24.14%) with repeated disease. Conclusions:JIA is a common rheumatic immune disease in children and all subtypes can be combined with lung injury, manifesting as interstitial lung disease mainly.The age and sex of JIA children combined with lung injury are not specific factors.The proportion of lung injury in SoJIA is significantly higher than that in other subtypes.SoJIA combined with macrophage activation syndrome can lead to respiratory failure, respiratory distress syndrome, and even death, which is one of the main factors leading to poor prognosis of JIA.HRCT is more sensitive to the diagnosis of lung injury.Lung function detection is a simple and easy method to evaluate and monitor lung injury.The prognosis of JIA children combined with lung injury is poor, which should be well concerned.