Sarcoidosis Masquerading as Atypical Hepatic Fibrosis in Takotsubo Cardiomyopathy

Abstract

Hepatic Sarcoidosis (HS) should be considered in patients presenting with atypical fibrosis. A 37-year old African American man with type-2 diabetes presented with sudden onset left flank pain and nausea. His vitals were within normal limits except sinus tachycardia of 102/minute and examination was benign. Laboratory data including transaminases were normal. CT abdomen revealed left ureteral calculus (UC) and unexplained massive hepatomegaly. He underwent lithotripsy for the UC and was scheduled for a liver biopsy (LB). During the LB, he developed ventricular tachycardia with a rate of 254/minute, which required immediate electro-cardioversion followed by placement of Automated Implantable Cardioverter-Defibrillator (AICD). A transthoracic echocardiogram showed ejection fraction of 25-30%, with apical akinesis suggestive of Takotsubo cardiomyopathy (TC). Meanwhile, the LB showed minimal necrotizing granulomatous inflammation with atypical fibrosis on trichome stain, negative for amyloid, acid fast and fungal organisms as well as IgG4. Sarcoidosis was suspected, however, workup including angiotensin converting enzyme (ACE) levels, calcium, liver enzymes, bilirubin was normal, with the lack of pulmonary involvement on Chest x-ray. Endomyocardial biopsy confirmed non-caseating granulomas typical for sarcoidosis. In retrospect, we made a diagnosis of hepatic sarcoidosis (HS) along with TC. HS is seen in 50-80% of systemic sarcoidosis with only 10-30% presenting with abnormal liver enzymes. African American ethnicity and splenomegaly are risk factors. Clinically, HS can manifest with nonspecific fatigue, fever, or specific symptoms like jaundice, pruritus, abdominal pain while long standing disease can result in portal hypertension and end-stage liver disease. Cholestasis and liver involvement manifests as an elevation of ALP and /or GGT. Normal ACE levels don't rule out sarcoidosis but may distinguish it from other granulomatous diseases. CT may show hepatomegaly to multiple hypodense liver nodules. Definitive diagnosis requires histopathological examination of liver biopsy showing noncaseating granulomas, which are negative for fungal stains and acid-fast mycobacteria. Medical management is indicated if symptomatic or having cholestasis or in those at high risk of hepatic complications. IV steroids (10-20 mg/day) and ursodeoxycholic acid (20-40 mg/day) are the first line, while second and third line drugs include immunosuppressants.