Case report: Progressive organ-threatening IgG4-related disease despite conventional immunosuppression: lessons from a 5-year longitudinal follow-up
6 affiliations
- 1Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College
- 2National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology
- 3Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education
- 4State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital
- 5Department of Endocrinology, Rheumatology and Immunology, Chengde Central Hospital
- 6Department of Rheumatology and Immunology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology
Abstract
Objective: To describe the 5-year clinical course of a patient who progressed from IgG4-related dacryoadenitis and sialadenitis (IgG4-DS) to IgG4-related hypophysitis (IgG4-RH) and retroperitoneal fibrosis, evaluating the clinical course and treatment response to conventional immunosuppressive therapy through longitudinal clinical, serological, endocrine, and magnetic resonance imaging (MRI) assessments.
Methods: Clinical records were retrospectively reviewed, focusing on disease progression after treatment discontinuation, responses to sequential conventional synthetic disease-modifying antirheumatic drugs (csDMARDs), and long-term pituitary endocrine outcomes.
Results: During 5 years of follow-up, a 44-year-old man with IgG4-DS developed partial anterior hypopituitarism, central diabetes insipidus, and retroperitoneal fibrosis following two episodes of unplanned treatment discontinuation. Serial MRI demonstrated progressive pituitary stalk thickening accompanied by heterogeneous enhancement. The maximum stalk diameter was 4.0 mm at diagnosis (May 2023), decreased to 3.6 mm during maintenance therapy (September 2024), increased to 8.5 mm at relapse (March 2025), and decreased to 7.0 mm after treatment modification (November 2025). Glucocorticoids combined with sequential csDMARDs controlled acute inflammation but failed to prevent permanent gonadal and posterior pituitary dysfunction. Serum IgG4 reached a peak of 38,100 mg/L, and its re-elevation preceded both clinical and radiological relapse. At the last assessment in January 2026, serum IgG4 remained elevated at 4,594 mg/L, with persistent hypogonadism and central diabetes insipidus.
Conclusions: Disease progression occurred mainly during treatment interruption, while residual activity persisted despite conventional csDMARDs. The relative contributions of treatment non-adherence and limited treatment efficacy remain uncertain. Combined serum IgG4 and MRI monitoring may aid early detection of relapse, but this requires validation in larger cohorts.
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BibTeX
@article{zhang2026case,
title = {{Case report: Progressive organ-threatening IgG4-related disease despite conventional immunosuppression: lessons from a 5-year longitudinal follow-up}},
author = {Xinyue Zhang and Xiaoyue Deng and Jiaxuan Li and Yanqiu Zhang and Shixiong Wei and Xiaofeng Zeng},
journal = {Frontiers in Immunology},
year = {2026},
month = oct,
doi = {10.3389/fimmu.2026.1975295},
url = {https://www.frontiersin.org/articles/10.3389/fimmu.2026.1975295/full},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Case report: Progressive organ-threatening IgG4-related disease despite conventional immunosuppression: lessons from a 5-year longitudinal follow-up
AU - Xinyue Zhang
AU - Xiaoyue Deng
AU - Jiaxuan Li
AU - Yanqiu Zhang
AU - Shixiong Wei
AU - Xiaofeng Zeng
PY - 2026
DA - 2026/10/28
JO - Frontiers in Immunology
DO - 10.3389/fimmu.2026.1975295
UR - https://www.frontiersin.org/articles/10.3389/fimmu.2026.1975295/full
AB - Objective: To describe the 5-year clinical course of a patient who progressed from IgG4-related dacryoadenitis and sialadenitis (IgG4-DS) to IgG4-related hypophysitis (IgG4-RH) and retroperitoneal fibrosis, evaluating the clinical course and treatment response to conventional immunosuppressive therapy through longitudinal clinical, serological, endocrine, and magnetic resonance imaging (MRI) assessments. Methods: Clinical records were retrospectively reviewed, focusing on disease progression after treatment discontinuation, responses to sequential conventional synthetic disease-modifying antirheumatic drugs (csDMARDs), and long-term pituitary endocrine outcomes. Results: During 5 years of follow-up, a 44-year-old man with IgG4-DS developed partial anterior hypopituitarism, central diabetes insipidus, and retroperitoneal fibrosis following two episodes of unplanned treatment discontinuation. Serial MRI demonstrated progressive pituitary stalk thickening accompanied by heterogeneous enhancement. The maximum stalk diameter was 4.0 mm at diagnosis (May 2023), decreased to 3.6 mm during maintenance therapy (September 2024), increased to 8.5 mm at relapse (March 2025), and decreased to 7.0 mm after treatment modification (November 2025). Glucocorticoids combined with sequential csDMARDs controlled acute inflammation but failed to prevent permanent gonadal and posterior pituitary dysfunction. Serum IgG4 reached a peak of 38,100 mg/L, and its re-elevation preceded both clinical and radiological relapse. At the last assessment in January 2026, serum IgG4 remained elevated at 4,594 mg/L, with persistent hypogonadism and central diabetes insipidus. Conclusions: Disease progression occurred mainly during treatment interruption, while residual activity persisted despite conventional csDMARDs. The relative contributions of treatment non-adherence and limited treatment efficacy remain uncertain. Combined serum IgG4 and MRI monitoring may aid early detection of relapse, but this requires validation in larger cohorts.
SN - 1664-3224
ER -