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
. 2014 Nov;24(6):382-6.
doi: 10.4103/0971-4065.133022.

IgG4 related renal disease: A wolf in sheep's clothing

A Rohan  1 , B Ravishankar  1 , S Vishwanath  1 , M Vankalakunti  2 , B Kishore  1 , H S Ballal  1
Affiliations
Case Reports

IgG4 related renal disease: A wolf in sheep's clothing

A Rohan et al. Indian J Nephrol. 2014 Nov.

Abstract

IgG4 related disease is a fibro-inflammatory condition with involvement of renal and extra renal organs, characterized by lymphoplasmacytic infiltration with organ dysfunction. We describe three cases of IgG4 related renal disease from a tertiary care hospital in south India.

Keywords: IgG4 related renal disease; interstitial nephritis; storiform fibrosis.

PubMed Disclaimer

Conflict of interest statement

Conflict of Interest: None declared.

Figures

Figure 1
Figure 1
Ultrasound showing focal hyperechoic tumefactive lesions in the kidneys with preserved kidney size
Figure 2
Figure 2
(a) Bland interstitial fibrotic process displacing the tubules wide apart (Periodic acid–Schiff, ×20), (b) Inflammatory infiltrates of dense plasma cells, eosinophils and lymphocytes (H and E, ×40)
Figure 3
Figure 3
(a) Expansile fibroinflammatory process with typical storiform pattern of fibrosis entrapping the obsolescent glomerulus and accompanied inflammatory cell components (MT, ×10), (b) Immunoperoxidase stain shows significant increase in IgG4+ cells, >40/hpf (×20)
See this image and copyright information in PMC

References

    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;38:982–4. - PubMed
    1. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366:539–51. - PubMed
    1. Stone JH, Khosroshahi A, Hilgenberg A, Spooner A, Isselbacher EM, Stone JR. IgG4-related systemic disease and lymphoplasmacytic aortitis. Arthritis Rheum. 2009;60:3139–45. - PubMed
    1. Dahlgren M, Khosroshahi A, Nielsen GP, Deshpande V, Stone JH. Riedel's thyroiditis and multifocal fibrosclerosis are part of the IgG4-related systemic disease spectrum. Arthritis Care Res (Hoboken) 2010;62:1312–8. - PubMed
    1. Saeki T, Saito A, Hiura T, Yamazaki H, Emura I, Ueno M, et al. Lymphoplasmacytic infiltration of multiple organs with immunoreactivity for IgG4: IgG4-related systemic disease. Intern Med. 2006;45:163–7. - PubMed

Publication types

LinkOut - more resources