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Published on: February 10, 2026
IgG4-related chronic tubulointerstitial nephritis without autoimmune pancreatitis and the time course of renal
Yutaka Tsubata1, Fumihiro Akiyama, Takeshi Oya
1Department of Internal Medicine, Niigata Prefectural Central Hospital, Joetsu. tsubata.y@gmail.com
Insights
This case highlights chronic immunoglobulin G4-related tubulointerstitial nephritis (TIN) in an elderly man with progressive kidney insufficiency. Early diagnosis and treatment with oral prednisolone can significantly improve renal function.
Area of Science:
- Nephrology
- Immunology
- Pathology
Background:
- Chronic kidney disease presents diagnostic challenges.
- Tubulointerstitial nephritis (TIN) can have various underlying causes.
- Elevated immunoglobulin G4 (IgG4) levels may indicate specific pathologies.
Observation:
- An elderly male patient presented with a gradual 18-month increase in serum creatinine from 0.9 to 5.6 mg/dL.
- The patient exhibited a high serum immunoglobulin G4 (IgG4) concentration.
- Renal biopsy revealed significant IgG4-positive plasma cell infiltration in the renal interstitium without glomerular pathology.
Findings:
- The diagnosis of IgG4-related tubulointerstitial nephritis (TIN) was established based on clinical and pathological findings.
- Treatment with oral prednisolone led to an immediate reduction in both serum creatinine and serum IgG4 levels.
Implications:
- IgG4-related TIN can manifest with chronic, slow progression over extended periods, even without overt urinary abnormalities.
- Clinicians should consider IgG4-related TIN in the differential diagnosis of chronic renal insufficiency of unknown etiology.
- Early recognition and treatment of IgG4-related TIN are crucial for preserving renal function.
Abstract:
We report an elderly man with chronic tubulointerstitial nephritis (TIN) showing a high serum immunoglobulin G4 (IgG4) concentration. His serum creatinine (Scr) level had gradually increased from 0.9 mg/dL to 5.6 mg/dL over 18 months. Renal biopsy showed marked IgG4-positive plasma cell infiltration in the interstitium without glomerular abnormalities and IgG4-related TIN was diagnosed. Oral prednisolone reduced his Scr and IgG4 levels immediately. The present case indicates that IgG4-related TIN can not only progress rapidly but also chronically over a long period without significant urinary abnormalities, and we should consider hidden IgG4-related TIN in cases of chronic renal insufficiency.
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