IgG4-related systemic diseases
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The IgG4-related systemic diseases, also IgG4-related diseases, are diseases characterized IgG4 positive plasma cells and fibrosis.[1]
- Lymphoplasmacytic sclerosing pancreatitis - a type of autoimmune pancreatitis.
- Retroperitoneal fibrosis.[2]
- Riedel's thyroiditis.
- Inflammatory pseudotumour,[2] also known as inflammatory myofibroblastic tumour (IMT); probably only a subset of IMT.[3]
- Sclerosing cholangitis[2] (IgG4-associated cholangitis[4]).
- Autoimmune pancreatitis (lymphoplasmacytic sclerosing pancreatitis).
- Others.
Microscopic
Features:[5]
- At least 10 IgG4 +ve plasma cells.†
- >40% of plasma cells IgG4 +ve.
> 10 in some organs including pancreas and >50 in most organs and a ratio of IgG4+ to IgG+ plasma cells of > 40%
Note: †Minimum number dependent on anatomical site.[5]
References
- ↑ Khosroshahi, A.; Stone, JH. (Jan 2011). "A clinical overview of IgG4-related systemic disease.". Curr Opin Rheumatol 23 (1): 57-66. doi:10.1097/BOR.0b013e3283418057. PMID 21124086.
- ↑ 2.0 2.1 2.2 Kamisawa, T.; Okamoto, A. (Jul 2008). "IgG4-related sclerosing disease.". World J Gastroenterol 14 (25): 3948-55. PMID 18609677.
- ↑ Saab, ST.; Hornick, JL.; Fletcher, CD.; Olson, SJ.; Coffin, CM. (Apr 2011). "IgG4 plasma cells in inflammatory myofibroblastic tumor: inflammatory marker or pathogenic link?". Mod Pathol 24 (4): 606-12. doi:10.1038/modpathol.2010.226. PMID 21297584.
- ↑ Barnabas, A.; Chapman, RW. (Feb 2012). "Primary sclerosing cholangitis: is any treatment worthwhile?". Curr Gastroenterol Rep 14 (1): 17-24. doi:10.1007/s11894-011-0230-8. PMID 22124849.
- ↑ 5.0 5.1 Abraham M, Khosroshahi A (September 2017). "Diagnostic and treatment workup for IgG4-related disease". Expert Rev Clin Immunol 13 (9): 867–875. doi:10.1080/1744666X.2017.1354698. PMC 5896560. PMID 28701054. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5896560/.