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'''IgG4-related systemic | The '''IgG4-related systemic diseases''', also '''IgG4-related diseases''', are diseases characterized IgG4 positive [[plasma cells]] and fibrosis.<ref name=pmid21124086>{{Cite journal | last1 = Khosroshahi | first1 = A. | last2 = Stone | first2 = JH. | title = A clinical overview of IgG4-related systemic disease. | journal = Curr Opin Rheumatol | volume = 23 | issue = 1 | pages = 57-66 | month = Jan | year = 2011 | doi = 10.1097/BOR.0b013e3283418057 | PMID = 21124086 }}</ref> | ||
==List of IgG4-related diseases== | |||
*Lymphoplasmacytic sclerosing pancreatitis - a type of autoimmune pancreatitis. | *Lymphoplasmacytic sclerosing pancreatitis - a type of autoimmune pancreatitis. | ||
*[[Retroperitoneal fibrosis]].<ref name=pmid18609677>{{Cite journal | last1 = Kamisawa | first1 = T. | last2 = Okamoto | first2 = A. | title = IgG4-related sclerosing disease. | journal = World J Gastroenterol | volume = 14 | issue = 25 | pages = 3948-55 | month = Jul | year = 2008 | doi = | PMID = 18609677 }}</ref> | *[[Retroperitoneal fibrosis]].<ref name=pmid18609677>{{Cite journal | last1 = Kamisawa | first1 = T. | last2 = Okamoto | first2 = A. | title = IgG4-related sclerosing disease. | journal = World J Gastroenterol | volume = 14 | issue = 25 | pages = 3948-55 | month = Jul | year = 2008 | doi = | PMID = 18609677 }}</ref> | ||
*[[Riedel's thyroiditis]]. | *[[Riedel's thyroiditis]]. | ||
*[[Inflammatory pseudotumour]].<ref name= | *[[Inflammatory pseudotumour]],<ref name=pmid18609677/> also known as ''inflammatory myofibroblastic tumour'' (''IMT''); probably only a subset of IMT.<ref name=pmid21297584>{{Cite journal | last1 = Saab | first1 = ST. | last2 = Hornick | first2 = JL. | last3 = Fletcher | first3 = CD. | last4 = Olson | first4 = SJ. | last5 = Coffin | first5 = CM. | title = IgG4 plasma cells in inflammatory myofibroblastic tumor: inflammatory marker or pathogenic link? | journal = Mod Pathol | volume = 24 | issue = 4 | pages = 606-12 | month = Apr | year = 2011 | doi = 10.1038/modpathol.2010.226 | PMID = 21297584 }}</ref> | ||
* | |||
*Sclerosing cholangitis<ref name=pmid18609677/> (IgG4-associated cholangitis<ref>{{Cite journal | last1 = Barnabas | first1 = A. | last2 = Chapman | first2 = RW. | title = Primary sclerosing cholangitis: is any treatment worthwhile? | journal = Curr Gastroenterol Rep | volume = 14 | issue = 1 | pages = 17-24 | month = Feb | year = 2012 | doi = 10.1007/s11894-011-0230-8 | PMID = 22124849 }}</ref>). | |||
*[[Autoimmune pancreatitis]] (lymphoplasmacytic sclerosing pancreatitis). | *[[Autoimmune pancreatitis]] (lymphoplasmacytic sclerosing pancreatitis). | ||
*Others. | *Others. | ||
==Microscopic== | |||
Features:<ref name=pmid28701054>{{cite journal |authors=Abraham M, Khosroshahi A |title=Diagnostic and treatment workup for IgG4-related disease |journal=Expert Rev Clin Immunol |volume=13 |issue=9 |pages=867–875 |date=September 2017 |pmid=28701054 |pmc=5896560 |doi=10.1080/1744666X.2017.1354698 |url=}}</ref> | |||
*At least 10 IgG4 +ve plasma cells.† | |||
*>40% of [[plasma cell]]s IgG4 +ve. | |||
Note: | |||
*† Minimum number dependent on anatomical site.<ref name=pmid28701054/> | |||
** Lymph node: 100 (or 50) plasma cells/HPF, where 1 HPF ~ 0.196 mm*mm and 3 HPFs (0.588 mm*mm) are assessed.<ref>{{cite journal |authors=Cheuk W, Chan JK |title=Lymphadenopathy of IgG4-related disease: an underdiagnosed and overdiagnosed entity |journal=Semin Diagn Pathol |volume=29 |issue=4 |pages=226–34 |date=November 2012 |pmid=23068302 |doi=10.1053/j.semdp.2012.07.001 |url=}}</ref> | |||
==Sign-out== | |||
===Not present=== | |||
<pre> | |||
The provided clinical history is noted. Plasma cells are not readily apparent (<10 plasma cells/0.196 mm*mm). | |||
</pre> | |||
==See also== | |||
*[[Plasma cells]]. | |||
*[[HPFitis]]. | |||
==References== | ==References== |
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