Mohammadreza Ardalan
1*, Kasra Ardalan
21 Kidney Research Center, Tabriz University of Medical Sciences, Tabriz, Iran
2 Faculty of Pharmacy, Eastern Mediterranean University, Famagusta, Turkish Republic of North Cyprus
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.