{"orpha":563581,"name":"Autoimmune hepatitis type 2","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["AIH type 2"],"definition":"A form of autoimmune hepatitis characterized by clinical presentation as cryptogenic hepatitis, interface hepatitis on histological examination, elevated serum aminotransferase levels, hypergammaglobulinemia/elevated immunoglobulin G, and presence of circulating autoantibodies, specifically antibodies to liver kidney microsome type 1 (anti-LKM1) and anti-liver cytosol type 1 (anti-LC1) antibodies. The disease typically manifests in childhood or adolescence with an acute onset, often with acute liver failure. Long-term immunosuppression is usually required. Reported concurrent autoimmune diseases are autoimmune thyroiditis, diabetes mellitus, and vitiligo.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-563581/","orphanet_url":"https://www.orpha.net/en/disease/detail/563581","names":{"fr":{"name":"Hépatite auto-immune type 2","synonyms":["HAI type 2"],"definition":"Forme d'hépatite auto-immune caractérisée par une présentation clinique à type d'hépatite d'étiologie inconnue, une hépatite d'interface à l'examen histologique, des taux sériques élevés d'aminotransférases, une hypergammaglobulinémie/des taux élevés d'immunoglobuline G et la présence d'auto-anticorps circulants, notamment les anticorps anti-LKM1 (liver kidney microsome type 1) et anti-LC1 (liver cytosol type 1). La maladie se déclare généralement pendant l'enfance ou l'adolescence, par une présentation aiguë souvent accompagnée d'une insuffisance hépatique aiguë. Un traitement immunosuppresseur à long terme est généralement nécessaire. Les maladies auto-immunes concomitantes rapportées sont la thyroïdite auto-immune, le diabète sucré et le vitiligo."},"de":{"name":"Hepatitis, autoimmune, Typ 2","synonyms":["AIH Typ 2"],"definition":"Eine Form der Autoimmunhepatitis, die sich klinisch als kryptogene Hepatitis manifestiert und histologisch durch eine interstitielle Hepatitis, erhöhte Serum-Aminotransferasewerte, Hypergammaglobulinämie/erhöhtes Immunglobulin G und zirkulierende Autoantikörper, insbesondere gegen das hepatozelluläre Mikrosom Typ 1 (Anti-LKM1) und das hepatozelluläre Cytosol Typ 1 (Anti-LC1), gekennzeichnet ist. Die Erkrankung manifestiert sich typischerweise im Kindes- oder Jugendalter mit einem akuten Schub, häufig mit akutem Leberversagen. In der Regel ist eine langfristige Immunsuppression erforderlich. Über begleitende Autoimmunerkrankungen wie Autoimmunthyreoiditis, Diabetes mellitus und Vitiligo wird berichtet."},"es":{"name":"Hepatitis autoinmune tipo 2","synonyms":["HAI tipo 2"],"definition":"Es una forma de hepatitis autoinmune caracterizada por una presentación clínica que se manifiesta como hepatitis criptogénica, hepatitis de interfase en el examen histológico, elevación de los niveles séricos de aminotransferasas, hipergammaglobulinemia/ inmunoglobulina G elevada, y presencia de autoanticuerpos circulantes, específicamente anticuerpos antimicrosomales hígado-riñon tipo 1 (anti-LKM1) y anticuerpos frente a la proteína citosólica hepática tipo 1 (anti-LC1). La enfermedad se manifiesta típicamente en la infancia o en la adolescencia con un inicio agudo, a menudo con insuficiencia hepática aguda. Suele ser necesario instaurar un tratamiento inmunosupresor a largo plazo. Las enfermedades autoinmunes concomitantes son la tiroiditis autoinmune, la diabetes mellitus y el vitíligo."},"it":{"name":"Epatite autoimmune tipo 2","synonyms":["EA tipo 2"],"definition":"Si tratta di una forma di epatite autoimmune caratterizzata, dal punto di vista clinico, da epatite criptogenetica, epatite di interfaccia all'esame istologico, livelli elevati di aminotransferasi nel siero, ipergammaglobulinemia/livelli elevati di immunoglobuline G, e presenza di autoanticorpi circolanti, in particolare anticorpi anti-microsomi epatici e renali di tipo 1 (anti-LKM1) e anticorpi anti-citosol epatico di tipo 1 (anti-LC1). In genere la malattia si manifesta nell'infanzia o nell'adolescenza, spesso con insufficienza epatica acuta. Di solito è necessaria un'immunosoppressione a lungo termine. Le malattie autoimmuni concomitanti comprendono la tiroidite autoimmune, il diabete mellito e la vitiligine."},"nl":{"name":"Auto-immune hepatitis type 2","synonyms":["AIH type 2"],"definition":"Een vorm van auto-immune hepatitis, gekenmerkt door klinische presentatie als cryptogene hepatitis, grensvlak-hepatitis bij histologisch onderzoek, verhoogde gehaltes van aminotransferasen in serum, hypergammaglobulinemie/verhoogd immunoglobuline G, en aanwezigheid van circulerende auto-antilichamen, vooral antilichamen tegen lever-nier microsoom type 1 (anti-LKM1) en lever-cytosol type 1 (anti-LC1). De ziekte manifesteert zich doorgaans in de kindertijd of adolescentie met een acute aanvang, vaak met acuut leverfalen. Langdurige immuunsuppressie is meestal vereist. Gelijktijdige auto-immuunziekten die worden gerapporteerd, zijn auto-immune thyreoïditis, diabetes mellitus, en vitiligo."},"pt":{"name":"Hepatite autoimune tipo 2"},"pl":{"name":"Autoimmunizacyjne zapalenie wątroby typu 2","synonyms":["AIH type 2"]},"cs":{"name":"Autoimunitní hepatitida typu 2","synonyms":["AIH type 2"]},"uk":{"name":"Аутоімунний гепатит, тип 2","synonyms":["AIH тип 2"],"definition":"A form of autoimmune hepatitis characterized by clinical presentation as cryptogenic hepatitis, interface hepatitis on histological examination, elevated serum aminotransferase levels, hypergammaglobulinemia/elevated immunoglobulin G, and presence of circulating autoantibodies, specifically antibodies to liver kidney microsome type 1 (anti-LKM1) and anti-liver cytosol type 1 (anti-LC1) antibodies. The disease typically manifests in childhood or adolescence with an acute onset, often with acute liver failure. Long-term immunosuppression is usually required. Reported concurrent autoimmune diseases are autoimmune thyroiditis, diabetes mellitus, and vitiligo."},"zh":{"name":"自身免疫性肝炎2型","synonyms":["AIH type 2"]},"tr":{"name":"Otoimmün hepatit tip 2","synonyms":["AIH tip 2"]}},"xrefs":[{"source":"ICD-10","code":"K75.4","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"DB96.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"61111843"},{"source":"MONDO","code":"0021176","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4303163","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":57146,"name":"Rare hepatic disease"},"parents":[{"orpha":2137,"name":"Autoimmune hepatitis","classifications":[183,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adolescent","Adult","Childhood"],"prevalence":[],"phenotypes":null}