S6004 PLE: An Uncommon Cause of Recurrent Ascites

Introduction: Common causes of recurrent ascites include cirrhosis, heart failure, and malignancy. When these are ruled out, protein-losing enteropathy (PLE) is a rare diagnosis that should be on the differential. PLE is a condition in which excessive protein loss in the gastrointestinal tract exceeds the protein synthesis capacity of the liver, resulting in hypoproteinemia. Etiologies of PLE can include any alteration to gastrointestinal mucosa like inflammatory bowel disease or celiac disease, increased central venous pressure from lymphatic obstruction, cardiac disease, and malnutrition. The clinical presentation can vary depending on the underlying etiology, but most commonly includes ascites, peripheral edema, and diarrhea. Case Description/Methods: A 66-year-old man with a past medical history of congestive heart failure with reduced ejection fraction and recently diagnosed ascites presented to the emergency room for worsening shortness of breath and abdominal distension. One month prior, he was diagnosed with recurrent ascites suspected to be from liver cirrhosis. Computed tomography of the abdomen during this admission revealed severe ascites but no radiographic evidence of cirrhosis. Paracentesis was performed and 5 liters of ascitic fluid were removed. Serum albumin to ascites gradient (SAAG) was low at 0.8 g/dL, which is not suggestive of portal hypertension or right sided heart failure. Of note, a recent right heart catheterization revealed normal filling pressures. Cytology was negative for malignancy, and fungal and tuberculosis testing was negative. Additionally, hepatitis virus serologies were negative, 24-hour urine protein was not suggestive of nephrotic syndrome, and ascitic amylase and protein levels were low. Eventually, his 24-hour stool clearance of alpha-1 antitrypsin returned elevated at 91 mL/day. A stool clearance greater than 27 mL/day in patients without diarrhea or 56 mL/day in patients with diarrhea is diagnostic of PLE. The patient received 1 additional paracentesis before being discharged with a recommendation to maintain a high-protein diet. Discussion: This case illustrates an uncommon cause of recurrent ascites due to protein-losing enteropathy, which was initially incorrectly attributed to cirrhosis. Confirming the diagnosis is crucial for appropriate treatment. When diagnostic workup revealed a low SAAG, common etiologies of low SAAG ascites were ruled out. Markedly elevated stool alpha-1 antitrypsin levels confirmed the diagnosis of PLE, and a high protein diet was instituted.