Lupus-associated protein-losing enteropathy.

Fourteen cases of primary lupus-associated protein-losing enteropathy have now been reported in the English-language literature. These cases were reviewed to find any consistent pattern of presentation. Lupus-associated protein-losing enteropathy typically occurs in young women, and is characterized by the onset of profound edema and hypoalbuminemia. In many cases it is the first obvious manifestations of systemic lupus erythematosus. Diarrhea is present about 50% of the time, but steatorrhea is absent. Diagnosis of protein-losing enteropathy can be successfully made by radioisotopic studies or 24-hour stool alpha 1-antitrypsin clearance. A normal lymphocyte count, elevated serum cholesterol, and absence of lymphangiectasia on intestinal biopsy help distinguish lupus-associated protein-losing enteropathy from protein-losing enteropathies due to direct or indirect lymphatic obstruction. Normal endoscopy and mucosal biopsy can rule out protein loss due to mucosal disruption. Prognosis appears to be excellent with corticosteroids, although other immunosuppressive therapies have been successfully used. A typical and illustrative case is presented as a focal point for review and discussion.