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Published on: November 10, 2021
IgG4-related tubulointerstitial nephritis
Tasnim Momoniat1, Deepa Jacob2, Neelaveni Duhli2
1Renal Department, Hull Royal Infirmary, Hull, UK tasnim.momoniat@nhs.net.
A 67-year-old man with chronic kidney disease experienced acute kidney injury, diagnosed as IgG4-related disease. Treatment with glucocorticoids and azathioprine improved renal function, highlighting effective management strategies for this condition.
Area of Science:
- Nephrology
- Immunology
- Rheumatology
Background:
- A 67-year-old male with a history of chronic kidney disease presented with acute kidney injury.
- The patient had a 9-year history of steroid-sensitive arthritis, epigastric pain, and isolated submandibular gland enlargement.
Observation:
- Elevated eosinophil count, total serum protein, and immunoglobulin G4 (IgG4) levels were noted.
- Serum hypocomplementaemia was also observed.
- Renal biopsy revealed tubulointerstitial nephritis with lymphoplasmacytic infiltrates, fibrosis, and IgG4-positive plasma cells.
Findings:
- The clinical presentation and pathological findings led to a diagnosis of IgG4-related disease.
- Immunohistochemistry confirmed the presence of IgG4-positive plasma cells in the renal biopsy.
Implications:
- The case highlights the importance of recognizing IgG4-related disease in patients presenting with renal dysfunction and systemic symptoms.
- Treatment with glucocorticoids resulted in improved renal function.
- Successful transition to azathioprine demonstrated its utility as a steroid-sparing agent for managing IgG4-related disease.
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