Abstract
Purpose: A 37 year-old female with systemic lupus erythematosus (SLE) was discovered to have incidental esophageal varices during an endoscopy for dyspepsia. Her labs were normal, except alkaline phosphatase 135(U/L) and platelet count 137,000 (BIL/L). An abdominal MRI revealed seven focal hepatic masses, splenomegaly, no ascites and a patent portal vein. An ultrasound-guided core biopsy of the largest liver lesion was consistent with focal nodular hyperplasia without surrounding cirrhosis. However, given the patient's history of SLE, azathioprine use, and portal hypertension, suspicion for co-existent nodular regenerative hyperplasia (NRH) was raised. Her rheumatologist agreed to discontinue azathioprine. Her varices persisted despite three surveillance endoscopies with banding. To better manage her portal hypertension she underwent a surgical portacaval shunt (Fig 1). During surgery, portal vein pressure measurements and a liver wedge biopsy were performed. The pre-shunt portal vein gradient measured 20 mmHg. The wedge specimen demonstrated multiple hypercellular nodules centered around portal triads without fibrosis, confirming NRH (Fig 2). An endoscopy six weeks after shunting verified complete resolution of varices. NRH is an uncommon condition, but an important cause of non-cirrhotic portal hypertension. A core biopsy can be insufficient, therefore diagnosis of NRH may require a surgical biopsy. In this case, the etiology is likely due to underlying SLE and azathioprine use. Management includes treating underlying disorders and discontinuing offending agents. Patients with NRH have preserved liver function, thus morbidity is due to portal hypertension. In this case, portal hypertension was successfully managed with a surgical portacaval shunt.Figure 1: No Caption available.Figure 2: No Caption available.
Published Version
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