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E-Poster Submission Deadline
Please prepare and upload your E-Poster no later than March 14, 2026 11.59PM CET. After this date, you will no longer be able to prepare and upload your E-poster and it will not be displayed and accessible on the congress website.
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Abstract titles should be brief and reflect the content of the abstract.
IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory condition that can involve multiple organs. Renal involvement may manifest as tubulointerstitial nephritis or secondary lesions, and some patients require renal replacement therapy. We present a rare case of IgG4-RD in an elderly diabetic patient who required temporary hemodialysis and subsequently developed autoimmune pancreatitis following corticosteroid therapy.
A 76-year-old man with a history of atrial septal defect, diabetes mellitus, hypertension, dyslipidemia, hyperuricemia, and atrial fibrillation was admitted to a local hospital with progressive dyspnea and generalized edema. Laboratory evaluation revealed eGFR 16 mL/min/1.73m², markedly elevated free κ (858.7 mg/L), free λ (180.7 mg/L), free light chain ratio (4.75) and IgG4 (1898 mg/dL), along with proteinuria and hematuria. A bone marrow biopsy showed hypocellular marrow without evidence of plasma cell dyscrasia. For further evaluation, the patient was transferred to our institution.
Imaging demonstrated bilateral renal atrophy and right renal artery stenosis. A subsequent renal biopsy revealed dense plasma cell infiltration with storiform fibrosis, consistent with IgG4-RD superimposed on diabetic nephropathy. Prednisolone (35 mg/day) was initiated on hospital day 12. However, on day 14, the patient developed autoimmune pancreatitis. Despite adjustments to diuretic therapy, volume overload worsened, and hemodialysis was initiated on day 21. By day 29, pancreatic enzyme levels had normalized, allowing for stent placement to treat the right renal artery stenosis. Thereafter, the patient's renal function and blood pressure stabilized, and hemodialysis was discontinued on day 42. At the time of transfer for rehabilitation, he remained on prednisolone 30 mg/day, with IgG4 reduced to 488 mg/dL and eGFR 8.8 mL/min/1.73m².
This case highlights IgG4-RD presenting with severe renal tubulointerstitial lesions and vascular involvement in an elderly diabetic patient, ultimately requiring temporary dialysis support. Notably, the patient developed autoimmune pancreatitis shortly after the initiation of corticosteroid therapy. This report underscores that IgG4-RD may exhibit asynchronous multi-organ involvement, necessitating careful monitoring even after treatment has begun.