A Patient with Primary Biliary Cirrhosis

Citation: 

Pages 29 - 33

Authors: 

Marcus E. S. Mason, MD, FCCWS, FABHP, MABHP

Case Presentation
Ms. K, a 57-year-old Hispanic woman, presented to the Emergency Department with a chief complaint of weight loss. She had lost 40 pounds over the last three to four months. She reported decreased appetite for several months, along with persistent headache, generalized fatigue, yellowing skin, lighter stools, and darker urine for the last two weeks. Ms. K had a mild degree of nausea over the last two weeks but had never actually vomited. Her headaches were bitemporal without dizziness, auras, or photophobia, and were somewhat relieved with over-the-counter (OTC) medications (acetaminophen, naproxen). Subsequent to taking these medications, she would develop intermittent
epigastric pain. The patient denied fevers, chills, chest pain, frequency, urgency, or diarrhea.

Hypertension was the only significant chronic condition in her past medical history. Ms. K had a blood transfusion many years ago in another country (reason unknown) and pneumonia about 12 years earlier. Regular medications included hydrochlorothiazide 25 mg by mouth daily and the aforementioned OTC anti-inflammatories. She had no known drug allergies. Her mother had hypertension, her father diabetes, and a deceased sister had had lupus. Ms. K denied alcohol, tobacco, or recreational drug use. She had been working in a janitorial service for some time; this was the only chemical exposure evident, which was related to cleaning fluids.

On physical exam, the patient’s vital signs were normal and she was afebrile. She presented as a well-developed, well-nourished female. She was alert and oriented times three, with a nonfocal neurologic exam. Cardiovascular and pulmonary exams were unremarkable. Physical findings of note were mildly icteric sclera and, upon palpation of her abdomen, an enlarged liver 2 cm below the right costal margin. Ms. K had normoactive bowel sounds and no abdominal distension or ascites. Rectal examination was heme negative. Laboratory work-up was within normal limits (WNL) overall, except for elevated liver function tests (LFTs), as listed in Table I. Chest x-ray and kidney and upper bladder films were unremarkable. A urinalysis showed trace protein and moderate bilirubin. A bedside ultrasound of the right upper quadrant showed a liver measuring 15 cm with increased echogenicity, suggesting the presence of fatty infiltration but revealing no gallstones, sludge, a negative Murphy’s sign, and no intrahepatic or extrahepatic biliary dilation or pericholecystic fluid. The body of the pancreas was WNL, but its head and tail areas were obscured by overlying bowel gas.

Ms. K was admitted to the hospital, and an abdominal computed tomography (CT) scan was performed that showed changes consistent with liver cirrhosis. No masses were evident, and a hepatitis panel was negative. Follow-up hemoglobin and hematocrit showed mild anemia. Mean cell volume subsequently increased, indicating that the patient was likely dehydrated. Follow-up alanine aminotransferase had increased two days later. Lipase remained high, although it had come down to below 1000 mIU/mL. Ms. K was placed on intravenous fluids and designated nothing per mouth. Additional lab results are included in Table I.

Hepatology was consulted and recommended magnetic resonance cholangiopancreatography (MRCP) to rule out stones and/or obstructions, as well as ordering numerous serologic markers. The possibility of a transient gallstone having passed and caused an acute pancreatitis was considered. Prednisone 40 mg by mouth daily was initiated over the concern that this might be an autoimmune hepatitis.

Further lab tests (Table I) showed increased levels and titers of antinuclear antibodies (ANA) speckled pattern and anti-smooth muscle antibodies (immunoglobulin G [IgG]); positive antimitochondrial antibodies (AMA) and AMA-M2 subsets; and positive anti-Sjögren’s syndrome A and anti-Sjögren’s syndrome B.



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