eISSN: 2345-4202  
J Nephropharmacol. 2018;7(1):36-39.
doi:10.15171/npj.2018.08

Case Report

IgG4-related disease; a unifying entity  

Mohammadreza Ardalan 1 * , Kasra Ardalan 2

1 Kidney Research Center, Tabriz University of Medical Sciences, Tabriz, Iran
2 Faculty of Pharmacy, Eastern Mediterranean University, Famagusta, Turkish Republic of North Cyprus
*Corresponding author: Prof. Mohammadreza Ardalan, Email: ardalan34@yahoo.com

Abstract

IgG4-related disease (IgG4-RD) is characterized by dense lymphoplasmacytic infiltration of different organs and elevated serum IgG4 level. The disease involves different organs including pancreas, kidney, aorta, lung, thyroid, salivary and lacrimal glands. IgG4-RD mimics malignancies, infectious and inflammatory disorders. Many disorders previously regarded as isolated, single-organ diseases such as autoimmune pancreatitis type 1, Mikulicz disease, and Riedel thyroiditis are connected within the spectrum of IgG4-RD. Chronic interstitial nephritis (CIN) is the main type of renal involvement. Membranous nephropathy is another renal picture of IgG4-RD. Multiple, hypo-dense, cortical, round and wedge-shape lesions are the main renal ultrasound features. IgG4-RD during its inflammatory phase successfully responds to glucocorticoid therapy. Here we presented a case of IgG4-RD and then reviewed the existing literature.

Implication for health policy/practice/research/medical education:

Chronic interstitial nephritis is the main type of renal involvement in IgG4-related disease (IgG4-RD). Membranous nephropathy is another renal picture of IgG4-RD. Multiple, hypo-dense, cortical, round and wedge-shape lesions are the main renal ultrasound features.

Please cite this paper as: Ardalan M, Ardalan K. IgG4-related disease; a unifying entity. J Nephropharmacol. 2018;7(1):3-6. DOI: 10.15171/npj.2018.02. 

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Submitted: 09 May 2017

Accepted: 19 Aug 2017
First published online: 28 Aug 2017
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