IgG4-Related Disease with Multiple Pancreatic Cysts: a Case Report and Literature Review

Yahui LIU; Shengmin YANG; Jiayuan DAI; Chunlei LI; Shaoying WANG; Jin XU; Yueying MAO; Linyi PENG; Jingjing CHAI; Min SHEN

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IgG4-Related Disease with Multiple Pancreatic Cysts: a Case Report and Literature Review

VernacularTitle:IgG4相关性疾病合并胰腺多发囊肿一例并文献复习
Author: Yahui LIU ¹ ; Shengmin YANG ² ; Jiayuan DAI ³ ; Chunlei LI ³ ; Shaoying WANG ³ ; Jin XU ³ ; Yueying MAO ³ ; Linyi PENG ⁴ ; Jingjing CHAI ³ ; Min SHEN ³
Author Information

1. Department of Rare Diseases, State Key Laboratory of Complex, Severe, and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China;Department of Nephrology, Zhangzhou Affiliated Hospital of Fujian Medical University, Zhangzhou 363000, China;.
2. Department of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China;.
3. Department of Rare Diseases, State Key Laboratory of Complex, Severe, and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China;.
4. Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China;.
Publication Type:Journal Article
Keywords: IgG4-related disease; autoimmune pancreatitis; pancreatic cystic lesions
From: JOURNAL OF RARE DISEASES 2026;5(3):336-341
CountryChina
Language:Chinese
Abstract:
Immunoglobulin G4-related disease (IgG4-RD) is a rare systemic immune-mediated disorder characterized by infiltration of IgG4-positive plasma cells and multiorgan fibrosis. Among them, cases with multiple pancreatic cystic lesions as the prominent manifestation are relatively rare, and they are easily misdiagnosed as pancreatic tumors or pancreatitis in clinical practice. Herein, we report one patient with IgG4-RD admitted to Peking Union Medical College Hospital. The patient initially presented with multiple pancreatic cystic lesions and aneurysmal dilatation of the abdominal aorta, accompanied by markedly elevated serum IgG4 levels. Combined with pathological findings from submandibular gland biopsy, the diagnosis of IgG4-RD was ultimately established. After treatment with a regimen of glucocorticoids combined with cyclophosphamide, the patient′s condition improved. Meanwhile, we performed a literature review of cases with IgG4-RD-related pancreatic cystic lesions, aiming to improve clinicians′ understanding of this rare disease phenotype.