Primary Sjögren's syndrome with protein-losing gastroenteropathy: report of two cases

Protein-losing gastroenteropathy is a rare complication in autoimmune diseases, especially in Sjögren's syndrome. We report two cases of primary Sjögren's syndrome, one in a 37-year-old female and another in a 50-year-old female, both of whom presented with peripheral edema. Protein-losing gastroenteropathies of the stomach and small intestine in the first patient and of the small intestine only in the second patient were demonstrated by abdominal Tc-99m labeled albumin abdominal scintigraphy and pathologic findings. Results of gastrointestinal tract biopsies from both patients showed chronic inflammatory cell infiltrations without lymphangiectasis or vasculitis. The patients were successfully treated with corticosteroids. Results of follow-up Tc-99m labeled albumin scintigraphy were well correlated with clinical improvement and the increase in serum albumin. Sjögren's syndrome should be considered as a cause of protein-losing enteropathy. Tc-99m labeled albumin abdominal scintigraphy is helpful in diagnosing the condition, locating the protein-losing sites, and monitoring the treatment outcome, especially in cases where protein loss occurs in the stomach.