Application of clinico-radiologic-pathologic diagnosis of diffuse parenchymal lung diseases in children in China

Abstract

Diffuse parenchymal lung diseases in children (chDPLD) or interstitial lung diseases in children (chILD) represent a heterogeneous group of respiratory disorders that are mostly chronic and associated with high morbidity and mortality. However, the incidence of chDPLD is so low that most pediatricians lack sufficient knowledge of chDPLD, especially in China. Based on the clinico- radiologic- pathologic (CRP) diagnosis, we tried to describe (1) the characteristics of chDPLD and (2) the ratio of each constituent of chDPLD in China. Data were evaluated, including clinical, radiographic, and pathologic results from lung biopsies. We collected 25 cases of chDPLD, 18 boys and 7 girls with a median age of 6.0 years, from 16 hospitals in China. The most common manifestations included cough (n = 24), dyspnea (n = 21), and fever (n = 4). There were three cases of exposure-related interstitial lung disease (ILD), three cases of systemic disease-associated ILD, nineteen cases of alveolar structure disorder-associated ILD, and no cases of ILD specific to infancy. Non-specific interstitial pneumonia (n = 9) was the two largest groups. Conclusion: Non-specific interstitial pneumonia is the main categories of chDPLD in China. Lung biopsy is always a crucial step in the final diagnosis. However, clinical and imaging studies should be carefully evaluated for their value in indicating a specific chDPLD.