An Uncommon Cause of Generalized Edema: Protein-Losing Gastroenteropathy with Primary Sjögren’s Syndrome

Protein-losing gastroenteropathy (PLGE) is a rare etiology of generalized edema. It is caused by a loss of serum protein into the gastrointestinal tract, resulting in hypoproteinemia, generalized edema, ascites, pleural effusions and occasionally, pericardial effusions. PLGE is associated with various conditions, such as gastrointestinal malignancy, cardiac diseases, inflammatory bowel disease, autoimmune diseases, and various infections. Here, we report a 69-year-old woman with a presentation of generalized edema. PLGE was confirmed via a fecal alpha-1 antitrypsin clearance test. Sjögren’s syndrome was consequently diagnosed according to the symptoms, high level of anti-SSA/SSB antibodies, positive Schirmer’s test, sialoscintigraphy, and salivary gland biopsy. Remission was noted by treatment with intravenous methylprednisolone followed by oral prednisolone. A review of the literature was also performed to evaluate the clinical manifestations, treatment, and outcome. Nephrologists should consider PLGE as one of the different diagnoses of generalized edema if common causes are excluded.