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IgG4-related disease

The information provided on this page is for informational purposes only. The National Organization for Rare Disorders (NORD) does not endorse the information presented. The content has been gathered in partnership with the MONDO Disease Ontology. Please consult with a healthcare professional for medical advice and treatment.

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Disease Overview

A recently described mass-forming lesion that occurs in the pancreas, submandibular glands, lacrimal glands, lymph nodes, and hepatobiliary tract. It is characterized by the presence of marked tissue sclerosis and infiltration by numerous plasma cells. The plasma cells show immunohistochemical staining for IgG4 and the serum IgG4 levels are often increased.

Synonyms

  • IgG4-RD
  • IgG4-associated disease
  • IgG4-positive multiorgan lymphoproliferative syndrome
  • IgG4-related autoimmune disease
  • IgG4-related sclerosing disease
  • IgG4-related systemic disease
  • IgG4-related systemic sclerosing disease
  • IgG4-syndrome
  • Immunoglobulin G4-related sclerosing disease
  • hyper-IgG4 disease
  • multifocal fibrosclerosis
  • multifocal idiopathic fibrosclerosis
  • systemic IgG4-related plasmacytic syndrome
  • systemic IgG4-related sclerosing syndrome
GARD Disease Summary

The Genetic and Rare Diseases Information Center (GARD) has information and resources for patients, caregivers, and families that may be helpful before and after diagnosis of this condition. GARD is a program of the National Center for Advancing Translational Sciences (NCATS), part of the National Institutes of Health (NIH).

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Orphanet

Orphanet has a summary about this condition that may include information on the diagnosis, care, and treatment as well as other resources. Some of the information and resources are available in languages other than English. The summary may include medical terms, so we encourage you to share and discuss this information with your doctor. Orphanet is the French National Institute for Health and Medical Research and the Health Programme of the European Union.

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