A Novel Clinical Entity, IgG4-related Disease (IgG4RD): General Concept and Details

Overview

Journal Mod Rheumatol

Publisher Oxford University Press

Specialty Rheumatology

Date 2011 Sep 2

PMID 21881964

Citations 307

Authors

Hisanori Umehara

Kazuichi Okazaki

Yasufumi Masaki

Mitsuhiro Kawano

Motohisa Yamamoto

Takako Saeki

Shoko Matsui

Takayuki Sumida

Tsuneyo Mimori

Yoshiya Tanaka

Kazuo Tsubota

Tadashi Yoshino

Shigeyuki Kawa

Ritsuro Suzuki

Tsutomu Takegami

Naohisa Tomosugi

Nozomu Kurose

Yasuhito Ishigaki

Atsushi Azumi

Masaru Kojima

Shigeo Nakamura

Dai Inoue

Affiliations

Soon will be listed here.

Abstract

IgG4-related disease (IgG4RD) is a novel clinical disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells. IgG4RD may be present in a certain proportion of patients with a wide variety of diseases, including Mikulicz's disease, autoimmune pancreatitis, hypophysitis, Riedel thyroiditis, interstitial pneumonitis, interstitial nephritis, prostatitis, lymphadenopathy, retroperitoneal fibrosis, inflammatory aortic aneurysm, and inflammatory pseudotumor. Although IgG4RD forms a distinct, clinically independent disease category and is attracting strong attention as a new clinical entity, many questions and problems still remain to be elucidated, including its pathogenesis, the establishment of diagnostic criteria, and the role of IgG4. Here we describe the concept of IgG4RD and up-to-date information on this emerging disease entity.

Citing Articles

IgG4-RD-Associated Mikulicz Syndrome Without Classic Systemic Involvement-A Case Report.

Mendoza-Vargas L, Sevilla-Fuentes S, Bautista-Becerril B, Berthaud-Gonzalez B, Falfan-Valencia R, Felix-Martinez L J Clin Med. 2025; 14(3).

PMID: 39941629 PMC: 11818687. DOI: 10.3390/jcm14030958.

Recent Evidence of the Role of CD4 T Cell Subsets in IgG4-related Disease.

Kamekura R, Sakamoto H, Yajima R, Yamamoto K, Okuni T, Yamamoto M JMA J. 2025; 8(1):40-47.

PMID: 39926068 PMC: 11799721. DOI: 10.31662/jmaj.2024-0096.

Pharmacological Management of IgG4-Related Disease: From Traditional to Mechanism-Based Targeted Therapies.

Akiyama M, Alshehri W, Saito K, Takeuchi T, Kaneko Y Drugs Aging. 2025; 42(2):111-126.

PMID: 39755996 DOI: 10.1007/s40266-024-01172-3.

Clinical characteristics of IgG4-related retroperitoneal fibrosis in a cohort of 117 patients with idiopathic retroperitoneal fibrosis: a retrospective study.

Hu J, Jin Z, Yu Y, Min D, Cai Q, Gao J Clin Rheumatol. 2025; 44(2):757-766.

PMID: 39755817 DOI: 10.1007/s10067-024-07276-w.

A Rare Presentation of IgG4-Related Sinusitis With Chronic Nasal Obstruction and Headache: A Case Report and Literature Review.

Jung D, Choi J J Rhinol. 2025; 31(3):179-183.

PMID: 39744449 PMC: 11685913. DOI: 10.18787/jr.2024.00025.

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