A 53-year-old woman comes to the hospital with right upper-quadrant abdominal discomfort and fatigue for the last 8 weeks. She also complains of jaundice and generalized joint pain. She has no nausea or vomiting. Her past medical history is significant for hypothyroidism for which she takes levothyroxine. Family history includes hypertension and ulcerative colitis. The patient lives in Florida and has not traveled recently. She does not use tobacco, alcohol, or illicit drugs.
Her blood pressure is 166/90 mm Hg, pulse is 96/min, and respirations are 14/min. Physical examination shows pale skin and scleral icterus. Cardiopulmonary examination is within normal limits. There is tenderness to palpation in the right upper quadrant. The liver edge is palpable 3 cm below the costal margin. There is no ascites, splenomegaly, or lower-extremity edema.
Laboratory results are as follows:

Viral hepatitis antibody panel and HIV testing are negative. Right upper-quadrant ultrasound shows hepatomegaly with normal liver echo texture and no gallstones. The common bile duct measures 6 mm.
Which of the following is the best initial test for this patient?

Question

A 53-year-old woman comes to the hospital with right upper-quadrant abdominal discomfort and fatigue for the last 8 weeks.  She also complains of jaundice and generalized joint pain.  She has no nausea or vomiting.  Her past medical history is significant for hypothyroidism for which she takes levothyroxine.  Family history includes hypertension and ulcerative colitis.  The patient lives in Florida and has not traveled recently.  She does not use tobacco, alcohol, or illicit drugs.
Her blood pressure is 166/90 mm Hg, pulse is 96/min, and respirations are 14/min.  Physical examination shows pale skin and scleral icterus.  Cardiopulmonary examination is within normal limits.  There is tenderness to palpation in the right upper quadrant.  The liver edge is palpable 3 cm below the costal margin.  There is no ascites, splenomegaly, or lower-extremity edema.
Laboratory results are as follows:

Viral hepatitis antibody panel and HIV testing are negative.  Right upper-quadrant ultrasound shows hepatomegaly with normal liver echo texture and no gallstones.  The common bile duct measures 6 mm.
Which of the following is the best initial test for this patient?

Quizplus · Accepted Answer

11ec193a_9814_cbde_853d_0bb9e6a7915f_MD0002_00 This patient's clinical presentation suggests autoimmune hepatitis, a chronic hepatitis due to autoantibodies against hepatocytes. It is more common in women and usually presents at age 40-50 years. Patients may be asymptomatic, symptomatic with debilitating nonspecific symptoms (eg, fatigue, abdominal pain, itching, arthralgias), or present with acute liver failure. Patients may have coexisting autoimmune disorders such as type 1 diabetes mellitus, thyroiditis, celiac sprue, or ulcerative colitis. Diagnosis is based on clinical presentation, serologic markers, and exclusion of other forms of chronic liver disease. Autoantibodies associated with autoimmune hepatitis include the following: Anti-nuclear antibody (ANA)Anti-smooth muscle antibody (ASMA)Anti-liver/kidney microsomal antibody (LKM-1)Liver cytosol antibody (LC-1) Anti-nuclear antibody (ANA) Anti-smooth muscle antibody (ASMA) Anti-liver/kidney microsomal antibody (LKM-1) Liver cytosol antibody (LC-1) Patients can also have elevated serum globulin concentration (most often immunoglobulin G) and false-positive hepatitis C viral antibody tests (confirmed by negative hepatitis C virus RNA). Serum aminotransferases are usually significantly more elevated than bilirubin or alkaline phosphatase. (Choice A) Patients with alpha-1 antitrypsin deficiency usually present in the first 2 decades of life with liver disease (eg, chronic hepatitis, cirrhosis, hepatocellular carcinoma) and possible skin disease (eg, panniculitis). After the second decade of life, pulmonary symptoms predominate and include early-onset emphysema. (Choice B) Granulomatosis with polyangiitis (Wegener's) presents with sinopulmonary disease, renal disease, and systemic symptoms (eg, cutaneous and ocular manifestations). Diagnosis is confirmed by positive cytoplasmic antineutrophil cytoplasmic antibodies, anti-proteinase-3 antibodies, and/or tissue biopsy showing vasculitis. (Choice D) Atypical celiac disease may present with minor gastrointestinal symptoms, iron deficiency anemia, osteoporosis, arthritis, elevated serum aminotransferases, neurologic symptoms, or infertility. However, serum aminotransferases are usually not this elevated, and jaundice is usually not present. (Choice E) This patient's viral hepatitis antibody panel is negative and hepatitis C viral RNA assay is therefore not required. Patients with autoimmune hepatitis may have false-positive hepatitis C viral antibody tests due to elevated IgG levels. In these patients, hepatitis C viral RNA assay can be helpful in excluding false-positive hepatitis C antibody testing. Educational objective: Autoimmune hepatitis is a chronic hepatitis due to autoantibodies against hepatocytes and is more common in women and individuals age > 40. Patients may be asymptomatic, be symptomatic (eg, fatigue, abdominal pain, itching, arthralgias), or develop acute liver failure. Diagnosis is confirmed by antibody testing (anti-nuclear antibody, anti-smooth muscle antibody, and/or anti-liver/kidney microsomal antibody), elevated serum aminotransferases, histology, and exclusion of other chronic liver diseases. __________ References: Acute and acute severe (fulminant) autoimmune hepatitis. (http://www.ncbi.nlm.nih.gov/pubmed/23090425) Autoimmune hepatitis: A comprehensive review. (http://www.ncbi.nlm.nih.gov/pubmed/23218932)

A 53-Year-Old Woman Comes to the Hospital with Right Upper-Quadrant