Sarcoidosis is a systemic disorder of unknown etiology and is characterized by the formation of granulomas throughout various organs in the body. The most common form is pulmonary sarcoidosis, which affects 90% of patients; the second most common form is oculocutaneous sarcoidosis;1 and the third most common form is hepatic sarcoidosis, which affects 63% to 90% of patients.2 Although the liver is frequently involved in all forms of sarcoidosis, only a fraction of patients present with clinically evident liver disease.1 Approximately 20% to 30% of patients have abnormalities on liver function tests, whereas only about 1% of patients show evidence of portal hypertension and cirrhosis.3 In fact, in the English literature, there were 35 reported cases of portal hypertension due to sarcoidosis between 1949 to 2001, of which 16 of the patients had no evidence of cirrhosis.4
The diagnosis of sarcoidosis is usually made by a compilation of clinical signs and symptoms, imaging studies, and biopsies demonstrating noncaseating granulomas. This case report describes a patient who presented with portal hypertension and esophageal variceal bleeding secondary to sarcoidosis of the liver without cirrhotic changes.
A 47-year-old woman presented to the ED via emergency medical services with a 1-hour history of hematemesis and melena. The patient stated that she felt fatigued, nauseated, and light-headed, but had no pain or focal weakness. Her medical history was significant for pulmonary and renal sarcoidosis. She underwent a liver biopsy 1 week prior to presentation, with a 6-day hospitalization period, due to new ascites found on examination.
The patient’s vital signs at presentation were: blood pressure (BP), 72/56 mm Hg; heart rate (HR), 133 beats/min, respiratory rate, 24 breaths/min; and temperature, 97.0oF. Oxygen saturation was 99% on room air. Physical examination revealed an alert and oriented middle-aged woman in extremis who was vomiting dark-colored blood. The cardiac and pulmonary examination revealed no extraneous sounds; the abdominal examination showed ascites with a liver edge palpable 4 cm beneath the right costal margin. The patient had no scleral icterus, palmar erythema, spider angiomata, fetor hepaticus, caput medusa, cutaneous ecchymoses, or any other stigmata of cirrhosis.
Two large-bore peripheral intravenous (IV) catheters were placed and a massive blood transfusion protocol was initiated. Packed red blood cells (PRBCs) from the resuscitation-area refrigerator were infused immediately via a pressurized fluid warmer.
After consultation with gastroenterology and general surgery services, the patient was given 1 g ceftriaxone IV, 1 g tranexamic acid IV, 20 mcg desmopressin IV, 50 mcg octreotide IV, 40 mg pantoprazole IV, 8 mg ondansetron IV, 4 g calcium gluconate IV, and 100 mg hydrocortisone IV.
Throughout the patient’s first 10 minutes in the ED, she remained persistently hypotensive and continued to vomit. Since the patient’s sensorium was intact, the team quickly discussed goals of care with her. The patient’s wishes were for maximal life-sustaining therapy, including endotracheal intubation and chest compressions, if necessary.
After this discussion, the patient was given IV etomidate and rocuronium and was intubated using video-assisted laryngoscopy. Following intubation, she was sedated with an infusion of fentanyl and underwent orogastric tube placement to aspirate stomach contents. A total of 2.5 L of frank blood were drained from the patient’s stomach.
A size 9 French single lumen left-femoral central venous catheter also was placed, through which additional blood products were infused. The patient received a total of 28 U PRBCs, fresh frozen plasma, and platelets over a 3-hour period. During transfusion, the patient’s vital signs improved to a systolic BP ranging between 110 to 120 mm Hg and an HR ranging between 90 to 110 beats/min; she did not experience any further hypotensive episodes throughout her stay in the ED.
Laboratory studies were significant for metabolic acidosis, hyperkalemia, acute on chronic anemia, leukocytosis, and acute on chronic renal failure. Synthetic function of the liver and transaminases appeared normal (Table).
The patient’s hyperkalemia was treated with 1 g calcium chloride IV, 50 g dextrose IV, and 10 U regular insulin IV. A portable chest radiograph showed an appropriately positioned endotracheal tube, and an electrocardiogram revealed sinus tachycardia without signs of hyperkalemia. A computed tomography (CT) scan of the abdomen and pelvis from the patient’s recent hospitalization, 1 week prior to presentation, showed hepatomegaly, liver granulomas, ascites, and periportal lymphadenopathy (Figure 1).
A review of the patient’s recent liver biopsy and ascitic fluid analysis revealed noncaseating granulomas compressing the hepatic sinusoids, and a serum ascites albumin gradient greater than 1.1 g/dL, implying portal hypertension without cirrhosis. The surgical team attempted to place a Sengstaken-Blakemore tube, but the device could not be positioned properly due to the patient’s narrowed esophagus.
The ED nurses cleaned the patient, preserving her dignity; thereafter the patient’s adult children visited with her briefly before she was taken for an upper endoscopy, which was performed in the ED. The endoscopy revealed actively hemorrhaging esophageal varices at the gastroesophageal junction (Figure 2). The varices were treated with endoscopic ligation; the gastroenterologist placed a total of 11 bands, resulting in cessation of bleeding.
After the endoscopy, the patient was admitted to the medical intensive care unit (ICU). Approximately 1.5 hours after arriving at the ICU, she developed renewed hematemesis. Despite efforts to control bleeding and provide hemodynamic support, the patient died 1 hour later.