Protein Losing Enteropathy: Diagnosis and Management

INTRODUCTION Protein-losing enteropathy (PLE), sometimes referred to as protein-losing gastroenteropathy, is an unusual cause of hypoproteinemia and is characterized by the shedding of large quantities of protein from the gastrointestinal (GI) mucosa. PLE may result from a wide variety of etiologies and can be both a diagnostic and therapeutic challenge to the practicing gastroenterologist. The clinical presentation of PLE may also be complicated by micronutrient deficiencies related to the underlying etiology of the PLE. In some cases, we have noted significant vitamin deficiencies and deficiency of essential fatty acids complicating the care of these patients. Through the use of a case illustration, we will explore a practical approach to the evaluation and management of PLE. In early 2016, a 51 year-old woman presented to the GI clinic upon referral by a hematologist because of the development of progressive hypoalbuminemia (albumin 2.6 g/dL) which had been identified approximately 1 year earlier. She described one normal appearing stool per day, denied any GI complaints, and her physical examination was entirely normal. PLE is generally considered to be a rare condition; however, given a lack of systematic screening and a wide variety of causes of hypoalbuminemia, its prevalence is poorly understood. There are robust data describing an incidence of up to 18% among survivors of the Fontan procedure, used as treatment of the univentricular congenital heart; however, data are much more limited Protein losing enteropathy (PLE) is an uncommon etiology of hypoproteinemia. It is caused by protein loss from compromised gastrointestinal (GI) mucosa as a result of GI mucosal diseases, GI tract infections, as well as from disruptions of venous and lymphatic outflow. The prevalence of PLE is poorly understood given the wide variety of causes of both hypoalbuminemia and PLE, and due to a lack of systematic screening. The evaluation of a potential PLE patient includes a careful assessment for alternative causes of hypoalbuminemia and a measurement of GI tract protein loss. This review provides the clinician with diagnostic criteria, as well as management and nutrition support options.