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.

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