- Author: Sang Hoon Lee, Young Ok Kim, Eun Lee, In Seok Jeong, Yoo-Duk Choi, Hwa Jin Cho
- Date: 2018/10/01
- Journal: Journal of Thoracic Disease;10(10);E730
- PMID: 30505512
- PMCID: PMC6236153
- DOI: 10.21037/jtd.2018.09.150
Abstract
Chronic pneumonitis of infancy (CPI) is a rare interstitial lung disease (ILD) that can be fatal in some cases. It occurs in infants who initially appear well and then develop respiratory symptoms with hypoxemia and diffuse interstitial infiltrates (1). Recently, the development of molecular genetic techniques has been rapidly increasing the understanding of the cause of CPI, and mutations in related genes encoding proteins such as surfactant protein (SP)-B, SP-C and ATP-binding cassette protein family A3 (ABCA3) have been identified as a representative cause (2).
Here, we report a 10-month-old girl who presented with hypoxemia and diagnosed as CPI with mediastinal lymphadenopathy on computed tomography (CT). In many literatures, the findings in CPI have been described only focusing on the lungs, and mediastinal lymphadenopathy has never been reported in patients with CPI. To the best of our knowledge, this is the first report to describe a case of CPI with mediastinal lymphadenopathy correlating to disease progression.