Clinical analysis of 53 children with connective tissue disease complicated with interstitial lung disease / 中华实用儿科临床杂志

ABSTRACT

Objective:To investigate the clinical manifestations, characteristics of chest high-resolution computed tomography (HRCT), and prognosis of connective tissue disease (CTD) complicated with interstitial lung disease (ILD) in children.Methods:The clinical data of 53 children with CTD-ILD who were admitted to the Department of Rheumatology and Immunology, Affiliated Xi′an Children′s Hospital of Xi′an Jiaotong University from October 2013 to October 2019 were retrospectively analyzed, including clinical manifestations, blood gas analysis, chest HRCT and prognosis.Results:As for these 53 children with CTD-ILD, the ratio of male to female was 1.0∶1.4, the average age was (7.50±3.34) years, and the course of disease was 2.00 (0.85, 7.50) months.Among them, there were 25 cases (47.2%) of juvenile idiopathic arthritis (JIA), 15 cases (28.3%) of systemic lupus erythematosus (SLE), 11 cases of polymyositis / dermatomyositis (PM/DM) (20.7%), 1 case of overlap syndrome (OS) (1.9%), and 1 case of allergic granulomatosis with polyangiitis (AGPA) (1.9%). Although cough (39.6%) was the most common symptom of respiratory system in these children with CTD-ILD and fever(66.0%) was the most common symptom in the systemic features.Blood gas analysis appeared abnormal in 17 cases, including 10 cases of hypoxemia (18.9%) and 7 cases of type Ⅰ respiratory failure (13.2%). HRCT chest showed ground glass shadow, strip shadow, subpleural spot shadow, grid shadow, pleural thickening, consolidation shadow, nodular shadow and cystic low-density shadow, with the proportion of 52.8%, 26.4%, 22.6%, 18.9%, 11.3%, 7.5%, 1.9% and 1.9%, respectively; nonspecific interstitial pneumonia (NSIP)(39.6%) was the most common type of imaging classification.After the combined treatment with glucocorticoids, immunosuppressive agents and biological agents, HRCT chest showed remarkably improvement in 36 cases (67.9%), while no change in 8 cases (15.1%). A total of 75.0%(33 cases) of 44 cases were infected in the course of combined treatment.In addition, 9 cases (17.0%) died from acute respiratory distress syndrome (ARDS), among which 4 cases exacerbated to rapid progressive luge disease and 5 cases aggravated secondary ARDS due to infection.Conclusions:Only a small number of children with CTD-ILD have respiratory symptoms and signs.HRCT chest contributes to the early diagnosis of CTD-ILD, and its imaging manifestations are diverse.Blood gas analysis and HRCT chest play an important role in the disease evaluation and treatment planning.Moreover, it is the direction for further research to develop effective methods to prevent and control secondary infection so as to improve the survival rate and reduce the mortality rate during the active treatment of primary diseases.