Home   Chapter Home   Jobs   Conferences   Fellowships   Books




Reviewer: Nat Pernick, M.D. (see Reviewers page)
Revised: 1 December 2011, last major update November 2011
Copyright: (c) 2002-2011, PathologyOutlines.com, Inc.


● 6% of total IgG; least abundant of IgG subclasses
● IgG4 related sclerosing disease is a syndrome affecting various sites with elevated serum IgG4 and site-related lymphoplasmacytic infiltrates, increased IgG4+ plasma cells and sclerosis
● Common sites are head and neck [eosinophilic angiocentric fibrosis (possibly, Am J Surg Pathol 2011;35:701), idiopathic cervical (neck) fibrosis (Am J Surg Pathol 2010;34:1678), salivary glands (Am J Surg Pathol 2010;34:202)], liver (Mod Pathol 2009;22:1287), lung (Am J Surg Pathol 2009;33:1886, Am J Surg Pathol 2009;33:1450), pancreatobiliary region (sclerosing pancreatitis, N Engl J Med 2001;344:732) and systemic (Am J Surg Pathol 2010;34:1812)
● Other sites: abdominal aortic aneurysms (Am J Surg Pathol 2008;32:197), breast (Am J Surg Pathol 2009;33:1058), CNS (Am J Surg Pathol 2009;33:1249), kidney (Hum Pathol 2011 Aug 31 [Epub ahead of print]), lymph nodes (Am J Surg Pathol 2008;32:671, Mod Pathol 2011 Nov 11 [Epub ahead of print]), ureter (Hum Pathol 2011;42:1178)
● In ocular disease, associated lymphomas have numerous IgG4+ monotypic plasma cells (Am J Surg Pathol 2008;32:1159)


● Cytoplasmic staining

Uses by Pathologists

● Diagnose IgG4 related sclerosing diseases, including some cutaneous pseudolymphomas (Am J Surg Pathol 2009;33:1713), although presence of numerous IgG4+ plasma cells by itself is not specific (Mod Pathol 2011;24:1480)
● Differentiate IgG4 related sclerosing disease (high number and percentage of IgG4+ plasma cells in tissue ) from inflammatory myofibroblastic tumor (low number and percentage, Am J Surg Pathol 2009;33:1330, but see Mod Pathol 2011;24:606) or other lesions (Am J Surg Pathol 2008;32:1770, Hum Pathol 2010;41:643)

Micro images

Cholangitis explant-IgG4 related

Eye-MALT lymphoma with infiltration by IgG4+ plasma cells

Gastric tumor (left) and ulcer (right)-IgG4 related

Hepatic tumors-IgG4 related

Kidney-tubulointerstitial nephritis-IgG4 related

Lacrimal gland-IgG4 related

Liver (inflammatory pseudotumor with sclerosing cholangitis)-IgG4 related

Lymphadenopathy-IgG4 related

Pancreatitis-IgG4 related

Positive staining - disease

● Plasma cells in IgG4+ related sclerosing disorders

End of Stains > IgG4

This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.

All information on this website is protected by copyright of PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at copyrightPathOut@gmail.com with any questions (click here for other contact information).