National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

IgG4-related disease

Información en español


Other Names:
IgG4-related systemic disease; IgG4-syndrome; IgG4-associated disease; IgG4-related systemic disease; IgG4-syndrome; IgG4-associated disease; IgG4-related sclerosing disease; IgG4-related systemic sclerosing disease; IgG4-related autoimmune disease; IgG4-positive multiorgan lymphoproliferative syndrome; Hyper-IgG4 disease; Systemic IgG4-related plasmacytic syndrome; Systemic IgG4-related sclerosing syndrome; Multifocal fibrosclerosis; Multifocal idiopathic fibrosclerosis; Immunoglobulin G4-related sclerosing disease See More
Subtypes:
IgG4-related disease is an immune-mediated condition that can affect multiple organ systems.[1][2][3][4] Common features include IgG4-related autoimmune pancreatitis, swelling of or within an organ system (an inflammatory pseudotumor), salivary gland disease (which can lead to enlargement of the salivary glands), swollen lymph nodes (lymphadenopathy), skin manifestations, and symptoms consistent with allergies or asthma. The cause of IgG4-related disease is unknown. Some researchers believe that it may be an autoimmune or allergic disorder.[1][2] IgG4-related disease is usually treated with systemic steroids like prednisone. Immunosuppressive medications (azathioprine or mycophenolate mofetil) and biologics (rituximab or bortezomib) may be used in those who don't respond to steroids or for longer-term management. In severe cases, organ replacement therapy may be needed.[1][2][3][4] 
Last updated: 5/9/2016

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to IgG4-related disease . Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Patient Registry

  • The Autoimmune Registry supports research for IgG4-related disease by collecting information about patients with this and other autoimmune diseases. You can join the registry to share your information with researchers and receive updates about participating in new research studies. Learn more about registries.

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss IgG4-related disease . Click on the link to view a sample search on this topic.

Selected Full-Text Journal Articles

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

  1. Yung A. IgG4-related disease. DermNet NZ. October 4, 2015; http://www.dermnetnz.org/systemic/igg4-disease.html.
  2. Moulsopoulos HM, Fragoulis GE, Stone JH. Overview of IgG4-related disease. UpToDate. January 4, 2016; http://www.uptodate.com/contents/overview-of-igg4-related-disease.
  3. Stone JH. Treatment Approaches to IgG4-related Systemic Disease. Current Opinion in Rheumatology. 2011; 23(1):67-71. http://www.medscape.com/viewarticle/733608.
  4. Khosroshahi A et al.. International Consensus Guidance Statement on the Management and Treatment of IgG4-Related Disease. Arthritis & Rheumatology. July 2015; 67(7):1688-1699. http://onlinelibrary.wiley.com/doi/10.1002/art.39132/epdf.