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

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


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.  


IgG4-related tubulointerstitial nephritis; retroperitoneal fibrosis; kidney biopsy; immunosuppression

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Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012; 366:539-551.

Deshpande V, Zen Y, Chan JK, Eunhee EY, Sato Y, Yoshino T, et al. Consensus statement on the pathology of IgG4-related disease. 2012; 25:1181-1192.

Cheuk W, Chan JK. IgG4-related sclerosing disease: a critical appraisal of an evolving clinicopathologic entity. Adv Anat Pathol. 2010; 17:303-332.

Bateman AC, Deheragoda MG. IgG4‐related systemic sclerosing disease–an emerging and under-diagnosed condition. Histopathology. 2009; 55:373-383.

Moutsopoulos HM, Fragoulis GE, Stone JH. Overview of IgG4-related disease. In Schur PH, Romain PL, editors. UpToDate. Retrieved 23 February 2017, from http://www.uptodate.com/contents/overview-of-igg4-related-disease.

Wallace ZS, Deshpande V, Mattoo H, Mahajan VS, Kulikova M, Pillai S, et al. IgG4‐related disease: clinical and laboratory features in one hundred twenty‐five patients. Arthritis Rheumatol. 2015; 67:2466-2475.

Okazaki K, Uchida K, Koyabu M, Miyoshi H, Takaoka M. Recent advances in the concept and diagnosis of autoimmune pancreatitis and IgG4-related disease. J Gastroenterol. 2011; 46:277-288.

Saeki T, Nishi S, Imai N, Ito T, Yamazaki H, Kawano M, et al. Clinicopathological characteristics of patients with IgG4-related tubulointerstitial nephritis. Kidney Int. 2010; 78:1016-1023.

Raissian Y, Nasr SH, Larsen CP, Colvin RB, Smyrk TC, Takahashi N, et al. Diagnosis of IgG4-related tubulointerstitial nephritis. J Am Soc Nephrol. 2011; 22:1343-1352.

Vivas I, Nicolas AI, Velazquez P, Elduayen B, Fernandez-Villa T, Martinez-Cuesta A. Retroperitoneal fibrosis: typical and atypical manifestations. Br J Radiol. 2000; 73:214-222.

Salemis NS, Tsiambas E, Tsohataridis E. Pelvic confined idiopathic retroperitoneal fibrosis mimicking a large tumor. Adv Med Sci. 2009; 54: 296-298.

Khosroshahi A, Wallace ZS, Crowe JL, Akamizu T, Azumi A, Carruthers MN, et al. International consensus guidance statement on the management and treatment of IgG4-related disease. Arthritis Rheumatol. 2015; 67:1688-1699.

Khosroshahi A, Stone JH. Treatment approaches to IgG4-related systemic disease. Curr Opin Rheumatol. 2011; 23: 67-71.

DOI: https://doi.org/10.21804/20-1-1493


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