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Case Reports
. 2021 Jan-Dec:9:23247096211022487.
doi: 10.1177/23247096211022487.

Immunoglobulin G-4-Related Retroperitoneal Fibrosis

Syed Arqum Huda  1 , Sara Akram Kahlown  2 , Rachel Elder  3 , Viren Kaul  3 , David Landsberg  3
Affiliations
Case Reports

Immunoglobulin G-4-Related Retroperitoneal Fibrosis

Syed Arqum Huda et al. J Investig Med High Impact Case Rep. 2021 Jan-Dec.

Abstract

Retroperitoneal fibrosis is caused by the replacement of normal retroperitoneal tissue with fibrosis. The majority of the cases are idiopathic, but some secondary causes include malignancy, infection, drugs, and radiotherapy. Immunoglobulin G-4 (IgG-4) related disease is a relatively newer disease and one of the rarer causes of retroperitoneal fibrosis. It usually involves the pancreas, lungs, kidneys, aorta lacrimal and salivary glands, or extrapancreatic bile duct. Elevated serum IgG-4 is the biomarker of the disease and its levels correlate with disease activity. High-dose glucocorticoid is the treatment of choice.

Keywords: IgG-4-related disease; acute renal failure; dialysis; hyperkalemia; retroperitoneal fibrosis.

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Conflict of interest statement

Declaration of Conflicting Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Figure 1.
Figure 1.
(A) Coronal section of computed tomography of the abdomen without contrast done on initial presentation shows retroperitoneal fibrosis (red arrows). (B) Coronal section of computed tomography of the abdomen with contrast done 45 days later shows decreased size of retroperitoneal fibrosis (red arrows).
Figure 2.
Figure 2.
Plasma cells (black arrow) rich infiltrate with scattered lymphocytes and eosinophils in the background of fibroblastic connective tissue (hemoglobin and eosin, 10×).
Figure 3.
Figure 3.
CD-138 immunostaining (black arrow) highlighting plasma cell infiltrate. CD-138 is a highly specific marker for plasma cells (40×).
Figure 4.
Figure 4.
IgG-4 immunostaining (black arrow) with variable density of cells with >10/high-power field (40×).
See this image and copyright information in PMC

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