Difference between revisions of "IgG4-related systemic diseases"

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'''IgG4-related systemic disease''', also '''IgG4-related disease''', is a disease 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>
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>


There are several IgG4-related diseases:
==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>
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*[[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==
48,475

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