IgG4-related kidney disease: a rare cause of tubulointerstitial nephritis

Thadathilankal J John; Adriano Pellizon; Wiiliam D Bates; Mogamat-Yazied Chothia

doi:10.21804/20-1-1493

Thadathilankal J John Stellenbosch University
Adriano Pellizon Stellenbosch University
Wiiliam D Bates National Health Laboratory Services and Department of Anatomical Pathology, Stellenbosch University & Tygerberg Hospital, Cape Town
Mogamat-Yazied Chothia Stellenbosch University and Tygerberg Hospital http://orcid.org/0000-0002-9801-1300

Abstract

A 42-year-old black female was referred with unexplained renal failure. Initial radiological examination revealed a right adnexal inflammatory mass and bilateral hydronephrosis. The renal failure persisted despite the insertion of double J stents. A kidney biopsy showed a lymphoplasmacytic interstitial infiltrate that stained positive for IgG4. A diagnosis of IgG4-related tubulointerstitial nephritis (TIN) with pelvic-limited retroperitoneal fibrosis (RPF) was made and immunosuppressive treatment was initiated, with a good initial response to therapy. This report highlights the importance of considering RPF in cases of unexplained obstructive uropathy and of considering the diagnosis of IgG4-related disease when TIN is found in combination with other organ involvement.

Author Biographies

Thadathilankal J John, Stellenbosch University

Department of Medicine, Registrar.

Adriano Pellizon, Stellenbosch University

Division of Nephrology, Stellenbosch University and Tygerberg Academic Hospital, Cape Town, South Africa.

Senior Registrar (Nephrology)

Mogamat-Yazied Chothia, Stellenbosch University and Tygerberg Hospital

Department of Medicine, Divisions of General Medicine & Nephrology, Faculty of Medicine and Health Sciences, Stellenbosch University & Tygerberg Academic Hospital, Cape Town, South Africa, Nephrologist and Senior Lecturer

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