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Case Reports
. 2021 Nov 11;14(11):e241942.
doi: 10.1136/bcr-2021-241942.

IgG4-related tubulointerstitial nephritis

Tasnim Momoniat  1 , Deepa Jacob  2 , Neelaveni Duhli  2 , Tom Jorna  3
Affiliations
Case Reports

IgG4-related tubulointerstitial nephritis

Tasnim Momoniat et al. BMJ Case Rep. .

Abstract

A 67-year-old man was referred to the renal team following an episode of acute kidney injury on a background of chronic kidney disease. He had a 9-year history of steroid-sensitive arthritis, epigastric pain and isolated submandibular gland enlargement. He was noted to have a raised eosinophil count, total serum protein and total immunoglobulin G4 (IgG4) level as well as a serum hypocomplementaemia. A renal biopsy showed a tubulointerstitial nephritis with lymphoplasmacytic infiltrates, fibrosis and IgG4-positive plasma cells on immunohistochemistry. A diagnosis of IgG4-related disease was made based on clinical presentation and pathology. Renal function improved with glucocorticoids and the patient was successfully transitioned to azathioprine as a steroid-sparing agent.

Keywords: acute renal failure; ear; nose and throat/otolaryngology; renal medicine; rheumatology.

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Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
Histopathology of kidney biopsy. (A) Lymphoplasmacytic infiltrate of tubulointerstitium with periglomerular fibrosis (periodic acid-Schiff (PAS) stain, magnification: ×50). (B) Periglomerular fibrosis (PAS). (C) Lymphoplasmacytic infiltrate (PAS, magnification: ×400). (D) and (E) Prominent immunoglobulin G4 (IgG4) positive plasma cells infiltrating the tubulointerstitium (IgG4 immunohistochemistry).
See this image and copyright information in PMC

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