Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2025 Sep 25;14(19):6774.
doi: 10.3390/jcm14196774.

New Developments in the Treatment of IgG4-Related Disease: A Comprehensive Clinical Approach

Andrés González-García  1   2 , Grisell Starita-Fajardo  1 , David Lucena López  1 , María Pilar Iranzo Alcolea  1 , María López-Paraja  1 , Mercedes Peña-Rodríguez  1 , Francisco Lirola Sánchez  1 , María Sánchez  1 , Adrián Viteri-Noël  1 , Martin Fabregate-Fuente  1 , Mónica López-Rodríguez  1   2 , José Luis Calleja-López  1   2 , Luis Manzano Espinosa  1   2
Affiliations
Review

New Developments in the Treatment of IgG4-Related Disease: A Comprehensive Clinical Approach

Andrés González-García et al. J Clin Med. .

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is an uncommon fibro-inflammatory process characterized by the infiltration of tissues and organs and a typically dramatic response to glucocorticoids. Its relapsing-remitting course, multisystemic involvement, and variability in epidemiological and prognostic features pose a significant diagnostic challenge for clinicians. Despite their effectiveness in symptom relief, prolonged glucocorticoid use remains a challenge in IgG4-RD management, prompting the search for steroid-sparing alternatives. Although rituximab has recently demonstrated efficacy in the treatment of IgG4-RD, no consensus exists regarding the optimal maintenance regimen. The emergence of new B-cell-targeted therapies and other immunomodulators represents a promising step toward more personalized treatment approaches. In this review, we provide an updated and integrative overview of the emerging treatment strategies for IgG4-RD, highlighting future directions towards individualized management.

Keywords: IgG4-related disease; fibrosis; immune dysregulation; inebilizumab; obexelimab; rilzabrutinib.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflicts of interest.

References

    1. Stone J.H., Zen Y., Deshpande V. IgG4-Related Disease. N. Engl. J. Med. 2012;366:539–551. doi: 10.1056/NEJMra1104650. - DOI - PubMed
    1. Hamano H., Kawa S., Horiuchi A., Unno H., Furuya N., Akamatsu T., Fukushima M., Nikaido T., Nakayama K., Usuda N., et al. High Serum IgG4 Concentrations in Patients with Sclerosing Pancreatitis. N. Engl. J. Med. 2001;344:732–738. doi: 10.1056/NEJM200103083441005. - DOI - PubMed
    1. Stone J.H. IgG4-Related Disease: Nomenclature, Clinical Features, and Treatment. Semin. Diagn. Pathol. 2012;29:177–190. doi: 10.1053/j.semdp.2012.08.002. - DOI - PubMed
    1. Wallace Z.S., Miles G., Smolkina E., Petruski-Ivleva N., Madziva D., Cook C., Fu X., Zhang Y., Stone J.H., Choi H.K. Incidence, Prevalence and Mortality of IgG4-Related Disease in the USA: A Claims-Based Analysis of Commercially Insured Adults. Ann. Rheum. Dis. 2023;82:957–962. doi: 10.1136/ard-2023-223950. - DOI - PubMed
    1. Khosroshahi A., Wallace Z.S., Crowe J.L., Akamizu T., Azumi A., Carruthers M.N., Chari S.T., Della-Torre E., Frulloni L., Goto H., et al. International Consensus Guidance Statement on the Management and Treatment of IgG4-Related Disease. Arthritis Rheumatol. 2015;67:1688–1699. doi: 10.1002/art.39132. - DOI - PubMed

LinkOut - more resources