Autoimmune Disease Flashcards
What is primary biliary cirrhosis?
Chronic autoimmune inflammation and destruction of the small and medium bile ducts
Causing cholestasis which may lead to fibrosis, cirrhosis and portal hypertension
Signs/symptoms in PBC?
Incidental raised ALP Lethargy Sleepiness Pruritus Arthralgia Xanthoma Hepatosplenomegaly Signs of chronic liver disease Osteoporosis
Ix in PBC?
LFT: raised ALP and GGT, bilirubin Albumin low Raised prothrombin time AMA +ve ANA +ve
US - exclude extraheaptic cholestasis
Biopsy - not usually needed
Mx for PBC?
Pruritus: colestyramine, ursodeoxycholic acid Osteoporosis - bisphosphonate Hypercholesterolaemia - statin Vitamin ADEK supplementation Immunosuppression - prednisolone Liver transplantation
What is primary sclerosing cholangitis? Aetiology/RF?
Progressive cholestasis due to fibrosis and obliteration of the biliary ducts
Associated with male, HLA, IBD - UC
Clinical features of PSC
Pruritus, fatigue, ascending cholangitis, cirrhosis, hepatic failure
Ix in PSC
LFT - raised ALP, bilirubin, GGT pANCA +ve ANA +ve Raised IgM MRCP/ERCP Liver biopsy - fibrous obliterative cholangitis
Mx in PSC
Liver transplant UDCA Prednisolone Methotrexate ERCP
What is autoimmune hepatitis
Autoimmune destruction and inflammatino of the liver
Young, middle aged women
Clinical features of autoimmune hepatitis?
Fever malaise UrticariaAbdominal discomfort Myalgia Hepatomegaly Chronic liver disease signs GRadual jaundice
What Ix for autoimmune hepatitis?
Raised AST, ALT, ESR, ANA +ve, Smooth muscle antibody +ve, raised IgG Anaemia Low white cell count, and platelets Lvier biopsy - fibrosis MRCP
Mx for autoimmune hepatitis
Steroids - prednisolone
Azathioprine
UDCA
Liver transplantation - decompensated cirrhosis or failure to respond to medical therapy
Associations of autoimmune hepatitis
Pernicious anaemia DM Autoimmune haemolysis UC Autoimmune thyroiditis
