Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2019 May 16;9(2):49-54.
doi: 10.1159/000500296. eCollection 2019 May-Aug.

IgG4-Related Kidney Disease: A Curious Case of Interstitial Nephritis with Hypocomplementemia

Emmett Tsz Yeung Wong  1 , Manjari Lahiri  2 , Ming Teh  3 , Christopher Cheang Han Leo  1
Affiliations
Case Reports

IgG4-Related Kidney Disease: A Curious Case of Interstitial Nephritis with Hypocomplementemia

Emmett Tsz Yeung Wong et al. Case Rep Nephrol Dial. .

Abstract

IgG4-related kidney disease has been relatively newly recognized over the last two decades as a combination of an autoimmune and allergic disorder, with elevated serum IgG4 level and hypocomplementemia among its characteristic features. Here we report the case of a man with interstitial nephritis presenting with acute kidney injury and hypocomplementemia but normal serum IgG4 level and provide a literature review of IgG4-related kidney disease. This case highlights the importance of IgG4-related kidney disease as an important differential diagnosis in any patient presenting with a clinical syndrome mimicking acute interstitial nephritis with hypocomplementemia. A high index of suspicion with a low threshold for performing a native kidney biopsy would be paramount as patients do respond well to corticosteroid therapy.

Keywords: Acute interstitial nephritis; Acute kidney injury; Hypocomplementemia; IgG4-related kidney disease; Tubulointerstitial disease.

PubMed Disclaimer

Figures

Fig. 1
Fig. 1
a IgG4-positive plasma cells (arrow) on immunohistochemistry (×400). b Extensive inflammation composed predominantly of mononuclear cells including prominent plasma cells admixed with scattered neutrophils and eosinophils (periodic acid-Schiff, ×400). c Storiform pattern of interstitial fibrosis (Masson trichrome, ×100). d Zonal character of tubulointerstitial inflammation with relative un-involvement of another zone (e) (periodic acid-Schiff, ×100).
Fig. 2
Fig. 2
Trend of creatinine.
See this image and copyright information in PMC

References

    1. Saito T, Stone JH, Nakashima H, Saeki T, Kawano M. Japan: Springer; 2016. IgG4-related kidney disease.
    1. Kamisawa T, Zen Y, Pillai S, Stone JH. IgG4-related disease. Lancet. 2015 Apr;385((9976)):1460–71. - PubMed
    1. Hamano H, Kawa S, Horiuchi A, Unno H, Furuya N, Akamatsu T, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med. 2001 Mar;344((10)):732–8. - PubMed
    1. Kamisawa T, Funata N, Hayashi Y, Eishi Y, Koike M, Tsuruta K, et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol. 2003;38((10)):982–4. - PubMed
    1. Salvadori M, Tsalouchos A. Immunoglobulin G4-related kidney diseases: an updated review. World J Nephrol. 2018 Jan;7((1)):29–40. - PMC - PubMed

Publication types