Background: Interstitial lung disease (ILD) is a crucial issues which impact the prognosis of connective tissue diseased patients and can cause uncontrolled systemic disease activity. So this work aimed to describe lung functions, radiological abnormalities, and the lung biopsy findings in a group of childhood interstitial lung disease (chILD) caused by underlying connective tissue disease.
Methods: Retrospective analysis, diagnostic modalities of chILD in 40 CTD children (JSLE, JIA, JDM, JSS, JMCTD) were evaluated; clinical manifestation, chest imaging (x-ray, HRCT) and lung biopsy in selected cases. Moreover, Spirometer Pulmonary function testing (PFT), 6-minutes walk test (6MWT), and transthoracic echocardiography were also evaluated to assess severity and follow up.
Results: For ILD associated with juvenile CTD, HRCT was the gold standard for diagnosis and diminish need for lung biopsy with common finding was widespread irregular interlobular septal thickening, ground-glass attenuation, ILD severity showed statistical significant with pulmonary function test and 6-minute walk test parameter.
Conclusion: For early prediction and follow up of development of ILD in CTD children, it is recommend to do routine checks of PFT even in the lack of respiratory warning sign, while HRCT is the gold standard to confirm and specify diagnosis.