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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.

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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)
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