A Case of Systemic Lupus Erythematosus Presenting with Protein Losing Enteropathy / 대한류마티스학회지

ABSTRACT

Protein losing enteropathy (PLE) is characterized by the loss of protein into the gastrointestinal tract that results in hypoalbuminemia and generalized edema. PLE is associated with several clinical disorders, but it is a rare manifestation of systemic lupus erythematosus (SLE), and it may be the presenting manifestation of SLE. We report a patient with SLE presenting with PLE, in whom methylprednisolone pulse therapy was highly effective. A 29-year-old women was admitted to our hospital with generalized edema. Laboratory findings revealed hypoalbuminemia, hypercholesterolemia and antinuclear antibody 1160, speckled type. Mucosal biopsies of the duodenal bulb and terminal ileum revealed edema and dilated lymphatics with infiltration of chronic inflammatory cells. PLE was diagnosed by marked elevation of alpha-1 antitrypsin clearance in stool and abnormal radioactivity within small intestine on 99mTc-labeled human serum albumin scan. Hypoalbuminemia and generalized edema improved rapidly after methylprednisolone pulse therapy.