Clinical characteristics of connective tissue disease associated interstitial lung disease in children

Abstract

Objective: To elucidate the clinical features of connective tissue disease associated interstitial lung disease (CTD-ILD) in children. Methods: A total of 24 children diagnosed with CTD-ILD and treated in Department of Rheumatology and Immunology, Shanghai Children's Medical Center from January 2010 to December 2019 were included in the study. Their medical data including clinical features, lab test, imaging, pulmonary function test, treatment and outcome were analyzed statistically. Results: In these 24 patients, 4 were males and 20 females. Their main clinical presentations were cough (10/24, 42%), shortness of breath (10/24, 42%), tachycardia in quiet state (8/24, 33%), fatigue with activity (7/24, 29%), and fever (6/24, 25%). The main abnormal physical examination findings were moist rales (4/24, 17%) and acropachy (2/24, 8%). There were various abnormal findings in the patients' lab test. All the patients (100%) had pulmonary interstitial changes on chest CT. Eight patients had spirometry test, and all (100%) showed decreased diffusion capacity. After immunosuppressive treatment, 15 cases (63%) improved, 4 cases (17%) gave up or died, while 5 cases (21%) had no significant improvement on the imaging examination. Conclusions: The onset of pediatric CTD-ILD could be insidious. Early diagnosis and aggressive treatment may change the prognosis, but are both difficult and need further studies.