C linical C ase of the M onth
A 44-Year-Old Woman With Jaundice and Abdominal Pain
Shane G. Guillory, MD; Matthew D. Jordan, DO; Bradley M. Spieler, MD; Matthew L. Safley, DO; John J. Hutchings, MD; Leslie A. Saketkoo, MD; Fred A. Lopez, MD, FACP
INTRODUCTION Primary biliary cirrhosis (PBC) is an autoimmune disease of the liver. It is characterized by a T-cell-mediated attack of the small, intrahepatic bile ducts. The exact etiology is unknown, but it is thought to be a result of a combina- tion of genetic predisposition and environmental factors. 1 The vast majority of patients affected by PBC are women. 2 The natural history of the disease varies by patient, but it is generally thought to be a chronic, progressive disease that carries a high mortality rate if not treated definitively with liver transplantation. 3 The diagnosis of PBC is made with the combination of clinical findings and laboratory data, including antimitochondrial antibodies. The diagnosis can also be further confirmed with a liver biopsy. We present a case of a woman who presented with abdominal pain, jaundice, and significant weight loss and was diagnosed with PBC based on autoantibody testing and liver biopsy. We also present a review of the epidemiology, etiology, clinical findings, diagnosis, and treatment options for PBC. CASE PRESENTATION A 44-year-old woman with a past medical history of cholelithiasis, status-post cholecystectomy three years prior, presented to her primary care physician complaining of progressively worsening right-sided abdominal pain of two months duration. She described the pain as “heavy,” located throughout the right side of her abdomen, 5/10 in intensity, and progressively worsening throughout the day. She reported that the pain was exacerbated by lying on her right side. She did admit to occasional radiation to her back and flank on the right side. The pain was not associated with eating. She also denied any nausea, vomiting, diar- rhea, melena, hematochezia, dysphagia, decreased appetite, or pruritis. Review of systems was otherwise positive for a 35-pound, unintentional weight loss over the prior three months. After her primary care physician noted abnormali-
ties in her routine lab work, the patient was told to present to the emergency department for further workup. Vital signs at the time of presentation were only notable for a pulse of 100 beats per minute and a temperature of 99.0 degrees Fahrenheit. Head and neck examination revealed sublingual icterus, as well as scleral icterus bilaterally. Ab- dominal examination revealedmild tenderness to palpation in the epigastric region, as well as the right upper quadrant without rebound or guarding. AMurphy’s sign was absent. The liver edge was palpated at 12 cm below the right costal margin, and the spleen was palpated at 3 cm below the left costal margin. Spider angiomata were noted in two spots on the right forearm, one spot on the left lower leg, and one spot on the back. Palmar erythema was noted as well. Initial laboratory workup revealed an elevated total bilirubin of 4.7 mg/dl (<1.3 mg/dl), alkaline phosphatase of 795 U/L (20-120 U/L), GGT of 1,199 U/L (<55 U/L), AST of 318 U/L (<46 U/L), and ALT of 289 U/L (<46 U/L). Erythrocyte sedimentation rate was mildly increased at 22 mm/hr (0-20 mm/hr). Total cholesterol was 554 mg/ dl (<200 mg/dl), triglyceride level was 923 mg/dl (<150 mg/dl), and LDL level was 344 mg/dl (<130 mg/dl). An acute hepatitis panel was performed and was negative. An ammonia level was elevated at 72 Umol/L (9-35 Umol/L). Urine drug screen and salicylate levels were not abnormal. Imaging at the time of admission included a right up- per quadrant ultrasound that revealed an enlarged liver at approximately 25 cm in sagittal dimension with no focal hepatic abnormality, an enlarged spleen at approximately 15.7 cm in sagittal dimension, normal flow in the portal vein, and an enlarged 2.3 cm lymph node near the porta hepatis. The gallbladder was surgically absent. The com- mon bile duct was noted to be normal, and no masses or free fluid were appreciated. Computed tomography of the abdomen on the day of admission confirmed the findings of the abdominal ultrasound (Figures 1, 2). A routine chest radiograph revealed no acute cardiopulmonary process. A rheumatologic workup was pursued. While anti-
J La State Med Soc VOL 166 May/June 2014 129
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