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primaire biliaire cholangitis en/of primaire scleroserende cholangitis en auto-immuunhepatitis-overlapsyndroom (aandoening)
primaire biliaire cholangitis en/of primaire scleroserende cholangitis en auto-immuunhepatitis-overlapsyndroom
primaire biliaire cholangitis en/of PSC en auto-immuunhepatitis-overlapsyndroom
Primary biliary cholangitis and/or primary sclerosing cholangitis and autoimmune hepatitis overlap syndrome
Overlap syndrome of autoimmune liver disease
A rare hepatic disease characterized by the overlap of primary biliary cholangitis and/or primary sclerosing cholangitis with autoimmune hepatitis, defined by the presence of at least two of the three recognized biochemical, serological, and histological criteria of each disease. The onset of the overlapping diseases can be simultaneous or sequential, with a variable interval of up to several years. Age of onset, gender predisposition, and clinical phenotype vary between each of the diseases, and the clinical presentation ranges from asymptomatic disease or unspecific symptoms such as fatigue, arthralgia, and pruritus, to established cirrhosis and decompensation, or also acute, fulminant hepatitis and liver failure. Association with extrahepatic autoimmune diseases is common.
Id1230291009
StatusPrimitive
Associated morphologyinflammatoire morfologie
Finding sitestructuur van lever
Pathological processauto-immuunproces
Associated morphologyinflammatoire morfologie
Finding sitestructuur van galwegen
SNOMED CT to Orphanet simple map562639
SNOMED CT to ICD-10 extended map
TargetK75.4
RuleTRUE
AdviceALWAYS K75.4 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified