Protein-Losing Enteropathy: An Uncommon Presentation of Celiac Disease

Abstract

Introduction: Protein-losing enteropathy (PLE) is an abnormal loss of protein from the gastrointestinal tract resulting in hypoproteinemia, and is usually not a single disease, but an atypical manifestation of other diseases. The primary causes of PLE can be divided into 3 broad etiologies, including erosive mucosal disease, non-erosive, and disorders involving increased interstitial pressures. Celiac disease is a non-erosive gastrointestinal disorder associated with protein-losing enteropathy. Case Report: A 30-year-old white male with no significant past medical history presented with chronic diarrhea for 8 months and progressively worsening generalized swelling for 3 months. The diarrhea initially started while he was incarcerated, and was described as 7 episodes of loose watery, non-bloody stool per day. The diarrhea partially improved with fasting, and there was no abdominal pain. Physical examination was notable for bibasilar rales and significant pitting edema involving bilateral lower extremities, scrotum, anterior abdominal wall, and lower chest. Labs were significant for total protein of 4.3 g/dL, albumin of 1.0 g/dL, normal creatinine, and no proteinuria. On push enteroscopy, diffuse scalloping and fissures of the duodenum and proximal jejunum were visualized. Duodenal and jejunal biopsies showed marked villous blunting with increased intra-epithelial lymphocytes, an increase in lamina propria chronic inflammatory infiltrate, and hyperplasia of the Brunner glands compatible with celiac disease. Celiac serologies were also elevated, including antigliadin Ab IgA 105 U/mL, t-Transglutaminase IgA >100 U/mL, and endomysial Ab IgA positive. A diagnosis of celiac disease presenting as protein-losing enteropathy was made, and the patient was initiated on a gluten-free diet. Discussion: In a patient presenting with hypoproteinemia, protein-losing enteropathy should be suspected in the absence of proteinuria, liver disease, and malnutrition. Celiac disease should be considered as one of the underlying causes of PLE. The mechanism of protein loss in celiac disease is not well described; however, it is likely a combination of decreased absorptive surface area from villous atrophy, paracellular protein loss, and increased mucosal cell turn over. Treatment of PLE should be focused on targeting the underlying disease.