Diagnostic and Treatment Workup for IgG4-related Disease

Overview

Journal Expert Rev Clin Immunol

Specialty Allergy & Immunology

Date 2017 Jul 14

PMID 28701054

Citations 17

Authors

Mary Abraham

Arezou Khosroshahi

Affiliations

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Abstract

IgG4-Related Disease is a newly recognized condition which is increasingly diagnosed by practitioners due to improvement in clinical awareness. Men and women have been found to be affected by this disease in various organs, more commonly with involvement of the salivary and lacrimal glands as well as pancreas and liver. Areas covered: The diagnosis and management of this condition remain challenging as biomarkers and therapies are being investigated. Hallmark features on histology are still the gold standard for confirmation of diagnosis, whereas serum IgG4 level has been shown to be neither necessary nor sufficient for the diagnosis. Glucocorticoids remain the most effective initial management for this condition while there are limited clinical trials on the effectiveness of maintenance therapy. Expert commentary: This review serves as an update on approaches for diagnosis and management of IgG4-RD. Most of the known data in this field comes from retrospective cohort studies and expert consensus guidelines but new ongoing prospective studies, clinical trials and better understanding of the pathogenesis of this condition are promising.

Citing Articles

The Management of IgG4-Related Disease in Children: A Systematic Review.

Sapountzi E, Kotanidou E, Tsinopoulou V, Fotis L, Fidani L, Galli-Tsinopoulou A Children (Basel). 2025; 12(2).

PMID: 40003315 PMC: 11854391. DOI: 10.3390/children12020213.

Discriminative features of immunoglobulin G4-related disease (IgG4-RD) and associated autoimmune rheumatic diseases (ARDs) in a nationwide observational cohort: study from the Egyptian College of Rheumatology.

El-Saadany H, El-Saadany H, Tharwat S, Soliman W, Shereef S, Elhefny A Clin Rheumatol. 2025; 44(2):747-756.

PMID: 39751976 PMC: 11775065. DOI: 10.1007/s10067-024-07274-y.

Pulmonary manifestations, treatments and outcomes of IgG4-related disease-a systematic literature review.

Dragos C, Joseph C, Elwell H, Dey M, Kouranloo K Rheumatol Int. 2024; 44(10):1875-1886.

PMID: 38769126 PMC: 11393110. DOI: 10.1007/s00296-024-05611-7.

Therapeutic Uses of Rituximab and Clinical Features in Immunoglobulin G4-Related Disease: A Systematic Review.

Patel U, Saxena A, Patel D, Ayesha I, Monson N, Klair N Cureus. 2023; 15(9):e45044.

PMID: 37701160 PMC: 10494991. DOI: 10.7759/cureus.45044.

Wang W, Kang X, Ding Y, Mao L, Dilinuer A, Li W Clin Cosmet Investig Dermatol. 2023; 16:1997-2004.

PMID: 37554302 PMC: 10404591. DOI: 10.2147/CCID.S406199.

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