II. Epidemiology

  1. Younger men most often affected
  2. Peak ages affected: 25-45 years old

III. Pathophysiology

  1. Biliary tree sclerosing inflammation and fibrosis
  2. Affects intrahepatic and extrahepatic bile ducts

IV. Associated Conditions

  1. Inflammatory Bowel Disease
    1. Ulcerative Colitis (60% to 70% co-Incidence)
    2. Crohn's Disease (rarely)
  2. AIDS
  3. Retroperitoneal fibrosis

V. Symptoms and Signs

VI. Labs: Liver Function Tests suggestive of cholestasis

  1. Bilirubin elevated
  2. Alkaline Phosphatase elevated

VII. Diagnosis: ERCP

  1. Stenosis and dilatation of biliary tract
  2. Intrahepatic and extrahepatic bile ducts affected

VIII. Differential Diagnosis

  1. Cholangiocarcinoma
  2. Caroli's Disease
    1. Cystic dilatation of bile ducts
  3. Fasciola hepatica
  4. Echinococcosis
  5. Ascariasis

IX. Course

  1. Chronic, slowly progressive
  2. Cholangitis
  3. May progress to Cirrhosis with Portal Hypertension

X. Management

  1. Pruritus
    1. Cholestyramine
  2. Bone preservation
    1. Vitamin D Supplementation
    2. Calcium Supplementation
  3. Biliary obstruction treatment
    1. Surgical drainage
  4. End stage Cirrhosis
    1. Liver transplantation

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Ontology: Cholangitis, Sclerosing (C0008313)

Definition (MSH) Chronic inflammatory disease of the BILIARY TRACT. It is characterized by fibrosis and hardening of the intrahepatic and extrahepatic biliary ductal systems leading to bile duct strictures, CHOLESTASIS, and eventual BILIARY CIRRHOSIS.
Concepts Disease or Syndrome (T047)
MSH D015209
ICD10 K83.0
SnomedCT 197443000, 235917005, 4032000
English Cholangiitides, Sclerosing, Cholangiitis, Sclerosing, Cholangitides, Sclerosing, Sclerosing Cholangiitides, Sclerosing Cholangiitis, Sclerosing Cholangitides, Cholangitis sclerosing, SCLEROSING CHOLANGITIS, Sclerosing cholangitis unspec, Sclerosing cholangitis unspecified, Cholangitis, Sclerosing [Disease/Finding], Cholangitis, Sclerosing, sclerosing cholangiitis, sclerosing cholangitis, chronic obliterative cholangitis, fibrosing cholangitis, Sclerosing Cholangitis, Sclerosing cholangitis unspecified (disorder), sclerosing cholangitis (diagnosis), Sclerosing cholangitis, Sclerosing cholangitis (disorder)
Dutch scleroserende cholangitis, cholangitis scleroserend, Cholangitis, scleroserende, Scleroserende cholangitis
German sklerosierende Cholangitis, Cholangitis sklerosierend, Cholangitis, sklerosierende
Swedish Kolangit, skleroserande
Japanese コウカセイタンカンエン, 硬化性胆管炎, 胆管炎-硬化性
Finnish Sklerosoiva kolangiitti
French CPS (Cholangite Primitive Sclérosante), Cholangite sclérosante, Cholangite primitive sclérosante, Angiocholite sclérosante
Russian KHOLANGIT SKLEROZIRUIUSHCHII, ХОЛАНГИТ СКЛЕРОЗИРУЮЩИЙ
Czech Sklerotizující cholangitida, Sklerózující cholangitida, cholangitida sklerózující, sklerozující cholangitida
Polish Zapalenie dróg żółciowych stwardniające
Hungarian Sclerotizáló cholangitis, Sclerotisaló cholangitis
Norwegian Kolangitt, skleroserende, Skleroserende kolangitt, Primær skleroserende kolangitt
Spanish colangitis esclerosante no especificada (trastorno), colangitis esclerosante no especificada, colangitis esclerosante (trastorno), colangitis esclerosante, Colangitis esclerosante, Colangitis Esclerosante
Portuguese Colangite Esclerosante Primária, Colangite esclerosante, Colangite Esclerosante
Italian Colangite sclerosante