Autoimmune Liver Diseases MCQs

This diagnosis is non-alcoholic fatty liver disease. The mildest type is simple fatty liver (steatosis), an accumulation of fat within the liver that usually causes no liver damage. This disease is usually nonprogressive and rarely causes liver cirrhosis. A potentially more serious type, nonalcoholic steatohepatitis (NASH), is associated with liver fibrosis. NAFLD is the most common chronic liver disease in many parts of the world, including the United States. Nonalcoholic fatty liver disease affects more women than men, and is associated with insulin resistance /diabetes and obesity . Reference : Harrison's Principles of Internal Medicine, 21 st Ed, Ch: 343Pg: 2621

IMNM, or autoimmune necrotizing myopathy, is characterized by the acute or insidious onset of symmetric, proximal more than distal weakness. Specific autoantibodies associated are: (anti-3-hydroxy-3methyl-glutaryl-coenzyme reductase [HMGCR] and anti-signal recognition particle [SRP]). Anti-HMGCR myopathy can be seen in patients receiving statins, inhibitors of HMGCR, particularly in patients aged >50 years. However, anti-HMGCR myopathy can develop in children and young adults without a history of statin use and can mimic a limb girdle muscular dystrophy. Reference: Harrison's Principles of Internal Medicine, 21 st ed, Ch: 365,: 2822

Numerous environmental events have been proposed to trigger the autoimmune process in genetically susceptible individuals; however, none have been conclusively linked to diabetes. Identification of an environmental trigger has been difficult because the event may precede the onset of DM by several years(Putative environmental triggers include viruses (coxsackie, rubella, enteroviruses most prominently), bovine milk proteins, nitrosourea compounds, vitamin D deficiency, and environmental toxins. Reference: Harrison's principles of Internal Medicine 21 st Ed, Ch: 403, Pg: 3100

Primary biliary cirrhosis is described. Liver transplantation does not cure the condition. Histology shows white cell damage to the biliary epithelium with non necrotising granuloma formation in the portal triad.Ursodeoxycholic acid lowers serum bilirubin and symptoms of itching, and prolongs the progression towards requirement for liver transplantation. IgM levels are particularly high in PBC. Desferrioxamine is used to treat hemochromatosis Prednisolone is used to treat autoimmune hepatitis Propranolol is for prophylaxis of upper GI bleed in patients of portal hypertension. Reference : Harrison's Principles of Internal Medicine 21 st ed, Ch: 244, Pg: 2627

It occurs frequently in young (10-20 years) and middle-aged women. Twenty five per cent present as acute hepatitis, but usually the onset is insidious.Some may be asymptomatic for years and then are found to have signs of chronic liver disease. Amenorrhoea is common. It is associated with hyperglobulinemia and other autoimmune diseases. Sixty per cent are associated with HLA-B8, DR3 and Dw3. Extrahepatic features include: arthritis Sjögren syndrome thyroiditis nephritis ulcerative colitis Coombs-positive hemolytic anemia. Reference :Harrison's Principles of Internal Medicine, 21 st ed, Ch:341 , Pg: 2613-2615

Ans. is 'a' i.e., Autoimmune hemolytic anemia Most common cause of spherocytes is immunehemolytic anemia.

Cross-reactivity between viral antigens (HCV NS3 and NS5A) and host autoantigens (cytochrome P450 2D6) explains the association between hepatitis C and autoimmune hepatitis. Rarely, autoimmune hepatitis occurs after acute hepatitis A and B. Reference: Harrison's Principles of Internal medicine, 21st edition page Ch: 341 Pg 2615

Primary sclerosing cholangitis presents at this age, there is cholestatic picture on the liver function tests, but not necessarily high bilirubin. pANCA is positive in about 90% of patients with PSC. A small proportion of patients (3%) will progress to cholangiocarcinoma at a later age. Reference : Harrison's Principles of Internal Medicine, 21 st ed, ch: 344, Pg: 2627-2628