A 63-year-old Hispanic woman, who was born and raised in the Dominican Republic, first presented to the emergency department with complaints of generalized weakness, fatigue, unintended weight loss, anorexia, progressively worsening abdominal pain, and fever of 5 days' duration. She had sought medical care in the Dominican Republic for similar symptoms 2 months before the index admission when she was found to have biliary obstruction due to compression from infiltrative liver lesions of unclear etiology. She underwent endoscopic retrograde cholangiopancreatography (ERCP) with placement of a plastic stent within the common bile duct (CBD) at the time. Later, the patient underwent a laparoscopic liver biopsy, and a diagnosis of granulomatous hepatitis secondary to tuberculosis (TB) was made. She refused anti-TB therapy. She subsequently moved to the United States and had an admission at another institution, a week before our index admission, during which the plastic stent within the CBD was exchanged with a full-covered metal stent. She also had a history of essential hypertension and diabetes mellitus. On examination, vital parameters were as follows: a temperature of 101.2 F, pulse rate of 107 beats/minute, blood pressure of 86/51 mmHg. The patient was confused, not able to follow commands. Precordial examination showed normal heart sounds with no murmur, rub, or gallop. Examination of lungs showed bilateral air entry with no adventitious sounds. Abdomen was soft, with firm hepatomegaly and diffuse abdominal tenderness. Patient underwent endotracheal intubation with initiation of mechanical ventilation, broad-spectrum intravenous antibiotics, and vasopressor therapy.
Complete blood count showed hemoglobin of 7.7 g/dL, white cell count of 24.9 K/muL, and platelet count of 188 K/muL. Comprehensive metabolic panel showed the following results: sodium 133 mEq/L, potassium 3.3 mEq/L, bicarbonate 18 mEq/L, chloride 94 mEq/L, glucose 185 mg/dL, and serum creatinine 3.8 mg/dL. Liver function tests revealed 231 international units (IU)/L of alanine transaminase, 447 IU/L of aspartate transaminase, 373 IU/L of alkaline phosphatase (ALP), and 2.4 mg/dL of total bilirubin with a direct fraction of 2.1 mg/dL. Additional workup done for evaluation of abnormal liver enzymes, including viral hepatitis panels (hepatitis A, B, and C), antinuclear antibody, anti-smooth muscle antibody, anti-liver-kidney microsomal antibodies, anti-mitochondrial antibody were negative. A computed tomography (CT) scan of the abdomen showed cholelithiasis, an ill-defined soft tissue density within the porta hepatis surrounding the pancreatic head, celiac axis, and common hepatic artery (Fig. 1A), along with multiple hypodense lesions within the liver and spleen (Fig. 1B).
The patient underwent an urgent ERCP that revealed an inwardly migrated metallic biliary stent. The CBD was cannulated through the biliary stent and balloon sweeps were performed with removal of sludge (Fig. 2A). Resolution of biliary obstruction with flow of dark-colored bile was noted. A subsequent occlusion cholangiogram did not reveal any intrahepatic biliary strictures or dilation (Fig. 2B). Hemodynamic status of the patient improved significantly post-ERCP, at which time point she was weaned off both vasopressor therapy and mechanical ventilation. Jaundice resolved and liver enzymes, barring ALP, gradually normalized. ALP remained persistently elevated at levels >250 IU/L. A CT-guided targeted needle biopsy of liver lesions revealed mild to moderate ductular reaction without cholestasis, broad areas of perivenular fibrosis, and scattered foci of lobular necroinflammation. No definitive diagnosis could be made with the biopsy specimen.
The patient was discharged from the hospital and was subsequently admitted a few weeks later for an elective laparoscopic cholecystectomy. Laparoscopy revealed multiple, discrete pale-colored well-circumscribed lesions in the hepatic parenchyma (Fig. 3A). A wedge biopsy of the liver lesion was performed at the same time. The biopsy specimen revealed numerous nonnecrotizing granulomas with epitheloid cells, multinucleated giant cells, and lymphocytes (Fig. 3B). Culture and staining for acid fast bacilli (AFB) were negative. Significant perivenular (zone 3) congestion and fibrosis were seen again (Fig. 3C). Patient was diagnosed as having extrapulmonary sarcoidosis with predominant hepatic involvement causing extrahepatic biliary obstruction and subsequent biliary sepsis.