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Review
. 2017 Sep;13(9):867-875.
doi: 10.1080/1744666X.2017.1354698. Epub 2017 Jul 24.

Diagnostic and treatment workup for IgG4-related disease

Mary Abraham  1 , Arezou Khosroshahi  1
Affiliations
Review

Diagnostic and treatment workup for IgG4-related disease

Mary Abraham et al. Expert Rev Clin Immunol. 2017 Sep.

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.

Keywords: IgG4; IgG4-RD; autoimmune; diagnosis and management; glucocorticoids; plasmablasts; rituximab.

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Conflict of interest statement

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Figures

Figure 1
Figure 1. Pathology of a lacrimal gland in a patient with multi systemic IgG4-RD
A: High power field view shows significant lymphoplasmacytic infiltration of the gland with fibrosis (Hematoxylin and Eosin staining) B: IgG4 immunohistochemical staining of the gland shows marked infiltration of the gland by IgG4 + plasma cells
Figure 1
Figure 1. Pathology of a lacrimal gland in a patient with multi systemic IgG4-RD
A: High power field view shows significant lymphoplasmacytic infiltration of the gland with fibrosis (Hematoxylin and Eosin staining) B: IgG4 immunohistochemical staining of the gland shows marked infiltration of the gland by IgG4 + plasma cells
Figure 2
Figure 2. Summary of key elements in diagnosis of IgG4-RD
*Gold Standard and still necessary for diagnosis
See this image and copyright information in PMC

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