• Qing Guo, Yue Ren, Quanyi Wang, Hongyun Pei, and Shenghua Jiang.
• College of Clinical Medicine, Jining Medical University, Jining, Shandong, China.
• Medicine (Baltimore). 2022 Jul 29; 101 (30): e29338e29338.

RationaleIgG4-related respiratory disease (IgG4-RRD) is a chronic autoimmune disease that affects the respiratory system and organs outside the respiratory system. This study explored the diagnosis and treatment of a case of IgG4-RRD with unilateral pleural effusion diagnosed using medical thoracoscopy, and provides an associated literature review. This report summarizes the clinical characteristics of IgG4-RRD involving the pleura to improve the diagnosis of this disease.Patient ConcernsA 39-year-old man presented with a 2-week history of cough and chest tightness. Both physical examination and imaging supported the presence of left pleural effusion.DiagnosisMedical electronic thoracoscopy was performed to obtain a pleural biopsy, which showed lymphoplasmacytic infiltration, 40 IgG4+ plasma cells per High Power Field (HPF) on microscopy, IgG4/IgG ratio >50%, phlebitis obliterans, and storiform fibrosis. The final diagnosis was IgG4-RRD.Interventions And OutcomesThe patient was treated with methylprednisolone, after which his symptoms improved, and he was discharged. Oral hormone therapy was continued outside the hospital. After 4 months, the patient returned to the hospital and his condition had improved significantly.LessonsPleural involvement in IgG4-RRD is rare, and its diagnosis depends on pleural biopsy. Thoracoscopy usually reveals pleural thickening, pleural nodules, and milky white plaques.Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.

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