Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
Age of Onset
Autosomal dominant ?A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease
Autosomal recessive ?Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype
X-linked dominant ?X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked recessive ?Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder
Mitochondrial or multigenic ?Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor ?Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
Other Names (AKA)
IgG4-related systemic disease; IgG4-syndrome; IgG4-associated disease;
IgG4-related disease is an immune-mediated condition that can affect multiple
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.
- DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
- 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.
- 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.
- Pieringer H, Parzer I, Wohrer A, Peis P, Oppl B, Zwerina J. IgG4-related disease: an orphan with many faces. Orphan Journal of Rare Diseases. 2014; 9:110.
- Yung A. IgG4-related disease. DermNet NZ. October 4, 2015; https://www.dermnetnz.org/systemic/igg4-disease.html.
- Moulsopoulos HM, Fragoulis GE, Stone JH. Overview of IgG4-related disease. UpToDate. January 4, 2016; https://www.uptodate.com/contents/overview-of-igg4-related-disease.
- Stone JH. Treatment Approaches to IgG4-related Systemic Disease. Current Opinion in Rheumatology. 2011; 23(1):67-71. https://www.medscape.com/viewarticle/733608.
- 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. https://onlinelibrary.wiley.com/doi/10.1002/art.39132/epdf.