• Hongmei Ren, Zhiqing Han, Xiaoming Zuo, Jinping Wang, Siming Meng, and Cuixia Zheng.
• Department of Respiratory Critical Medicine, Yangpu Hospital of Tongji University, Shanghai, China.
• Medicine (Baltimore). 2022 Nov 25; 101 (47): e32075e32075.

RationaleIgG4-related lung disease (IgG4-RLD) is an unusual disease, with various clinical manifestations and various chest imaging findings. The patients may have no respiratory symptoms. Therefore, diagnosis is challenging. This can easily cause misdiagnosis and mistreatment.Patient ConcernsA 71-year-old male presented with chest pain, cough, and shortness of breath. Plain chest computed tomography scans showed multi-locus nodes at the center of the hilum.DiagnosisPercutaneous lung biopsy was performed, and IgG4-RLD was diagnosed.InterventionsPrednisone was orally administered daily.OutcomesThe case's symptoms improved. The patient was discharged from the hospital. After 2 months of reexamination, his symptoms were relieved. Reexamination of the chest computed tomography showed that multi-locus nodes of the lung were obviously absorbed compared with those before.LessonsIgG4-RLD is a rare respiratory disease. It has atypical clinical manifestations and chest images. We report the first case of IgG4-RLD showing multi-locus nodes centered on the hilar, hypertrophic mucosa; as well as a narrow and even occluded lumen.Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.

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