CASE REPORT article
Front. Immunol.
Sec. Autoimmune and Autoinflammatory Disorders: Autoinflammatory Disorders
IgG4-Related Disease Presenting with Hypercalcemia: Case Report and Mechanistic Insights
Provisionally accepted
- 1Department of Nephrology, Institute of Kidney Diseases, West China Hospital, Sichuan University, Chengdu, Sichuan, China
- 2Department of Gastroenterology, West China Hospital, Sichuan University, Chengdu, Sichuan, China
- 3Department of Nephrology, Bozhou District People's Hospital of Zunyi City, Guizhou, China
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Immunoglobulin G4-related disease (IgG4-RD) is a rare, multisystem disorder with diagnostic challenges. We report a case of IgG4-RD initially presenting with acute kidney injury (AKI) and hypercalcemia in a 61-year-old woman with a history of recurrent eyelid edema. On admission, serum creatinine and calcium were significantly elevated (414 μmol/L and 3.69 mmol/L, respectively). After excluding other etiologies, diagnosis was confirmed by renal biopsy and elevated serum IgG4 levels. Treatment with prednisone acetate and supportive care resulted in gradual clinical and laboratory improvement. This case underscores the importance of considering IgG4-RD in patients with unexplained AKI and hypercalcemia, highlighting the value of early diagnosis and targeted therapy. Furthermore, we explored potential pathophysiological mechanisms underlying hypercalcemia in IgG4-RD and found that M2 macrophages may overexpress 1α-hydroxylase, enhancing the conversion to active 1,25(OH)₂D, and thereby increasing intestinal calcium absorption and leading to hypercalcemia, which has also been described in granulomatous diseases.
Keywords: IgG4-related disease (IgG4-RD), Hypercalcemia, Acute Kidney Injury, M2 macrophages, 1α-hydroxylase (CYP27B1)
Received: 08 Oct 2025; Accepted: 28 Nov 2025.
Copyright: © 2025 Li, Niu, Li and Hu. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Ling Li
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