Autoimmune hepatitis 660869 223952943 2008-07-06T17:51:26Z SmackBot 433328 Date the maintenance tags or general fixes {{Infobox_Disease | Name = Autoimmune hepatitis | Image = | Caption = | DiseasesDB = 1150 | ICD10 = {{ICD10|K|75|4|k|70}} | ICD9 = {{ICD9|571.49}} | ICDO = | OMIM = | MedlinePlus = 000245 | eMedicineSubj = med | eMedicineTopic = 366 | MeshID = D019693 | }} Anomalous presentation of [[human leukocyte antigen]] (HLA) class II on the surface of [[hepatocyte]]s,{{Fact|date=May 2008}} possibly due to [[gene]]tic predisposition or [[acute liver infection]]; causes a cell-mediated [[immune response]] against the body's own liver, resulting in '''autoimmune hepatitis'''. Autoimmune hepatitis has an incidence of 1-2 per 100,000 per year, and a prevalence of 10-20/100,000. As with most other [[autoimmune disease]]s, it affects [[women]] much more often than [[men]] (70%).<ref name="urlAutoimmune Hepatitis">{{cite web |url=http://www.cumc.columbia.edu/dept/gi/autoimmune.html |title=Autoimmune Hepatitis |format= |work= |accessdate=}}</ref> [[Liver enzyme]]s are elevated, as may be [[bilirubin]]. Autoimmune hepatitis can progress to [[cirrhosis]]. ==Treatment== Treatment is with [[steroid]]s and [[immunosuppressive]] drugs such as [[azathioprine]] and remission can be achieved in up to 60-80% of cases.<ref name="pmid8109584">{{cite journal |author=Krawitt EL |title=Autoimmune hepatitis: classification, heterogeneity, and treatment |journal=Am. J. Med. |volume=96 |issue=1A |pages=23S–26S |year=1994 |month=January |pmid=8109584 |doi= |url=}}</ref> ==Diagnosis== The [[diagnosis]] of autoimmune hepatitis is best achieved with a combination of [[clinical]], [[laboratory]] and histological findings. A number of specific [[antibodies]] found in the [[blood]] ([[antinuclear antibody]] (ANA), [[smooth muscle]] [[antibody]] (SMA), [[liver/kidney microsomal antibody]] (LKM-1), [[anti soluble liver antigen]] (SLA/LP) and [[anti-mitochondrial antibody]] (AMA)) are of use, as is finding an increased [[IgG|Immunoglobulin G]] level. However, the [[diagnosis]] of autoimmune hepatitis always requires a liver [[biopsy]]. In complex cases, a [[scoring system]] can be used to help determine if a patient has autoimmune hepatitis, which combines clinical and laboratory features of a given case.<ref name="pmid10580593">{{cite journal |author=Alvarez F, Berg PA, Bianchi FB, ''et al'' |title=International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis |journal=[[J. Hepatol.]] |volume=31 |issue=5 |pages=929–38 |year=1999 |month=November |pmid=10580593 |doi= |url=http://linkinghub.elsevier.com/retrieve/pii/S0168827899802979 |accessdate=2008-05-09}}</ref><ref name="urlAutoimmune Hepatitis Calculator">{{cite web |url=http://homepage.mac.com/sholland/contrivances/aihcalc.html |title=Autoimmune Hepatitis Calculator |format= |work= |accessdate=2008-05-09}}</ref> Overlapping presentation with [[primary biliary cirrhosis]] and [[primary sclerosing cholangitis]] has been observed.<ref name="pmid17486048">{{cite journal |author=Washington MK |title=Autoimmune liver disease: overlap and outliers |journal=[[Mod. Pathol.]] |volume=20 Suppl 1 |issue= |pages=S15–30 |year=2007 |month=February |pmid=17486048 |doi=10.1038/modpathol.3800684 |url=http://dx.doi.org/10.1038/modpathol.3800684}}</ref> ==Classification== Four subtypes are recognised, but the clinical utility of distinguishing subtypes is limited. # positive ANA and SMA,<ref name="pmid18528935">{{cite journal |author=Bogdanos DP, Invernizzi P, Mackay IR, Vergani D |title=Autoimmune liver serology: Current diagnostic and clinical challenges |journal=World J. Gastroenterol. |volume=14 |issue=21 |pages=3374–3387 |year=2008 |month=June |pmid=18528935 |doi= |url=http://www.wjgnet.com/1007-9327/14/3374.asp}}</ref> raised [[immunoglobulin G]] (classic form, responds well to low dose steroids); # positive [[LKM-1]] (typically female children and teenagers; disease can be severe); # positive antibodies against [[soluble liver antigen]]<ref name="urlautoimmune hepatitis">{{cite web |url=http://www.meddean.luc.edu/Lumen/MedEd/orfpath/autoimmune%20hepatitis.htm |title=autoimmune hepatitis |format= |work= |accessdate=}}</ref> (this group behaves like group 1)<ref name="urlMedscape & eMedicine Log In">{{cite web |url=http://www.medscape.com/viewarticle/445619_14 |title=Medscape & eMedicine Log In |format= |work= |accessdate=}}</ref> # no autoantibodies detected (~20%){{Fact|date=July 2008}} ==References== {{reflist}} {{Gastroenterology}} [[Category:Gastroenterology]] [[nl:Auto-immuun hepatitis]]