Table of Contents
Definition / general | Interpretation | Uses by pathologists | Microscopic (histologic) images | Positive staining - diseaseCite this page: Pernick N. IgG4. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/stainsIgG4.html. Accessed December 25th, 2024.
Definition / general
- 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;43:536), lymph nodes (Am J Surg Pathol 2008;32:671, Mod Pathol 2011;25:480), ureter (Hum Pathol 2011;42:1178)
- In ocular disease, associated lymphomas have numerous IgG4+ monotypic plasma cells (Am J Surg Pathol 2008;32:1159)
Interpretation
- 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)
Microscopic (histologic) images
Positive staining - disease
- Plasma cells in IgG4+ related sclerosing disorders