Primary protein-losing enteropathy in anti-double-stranded DNA disease: the initial and sole clinical manifestation of occult systemic lupus erythematosus?

Hypoalbuminemia and generalized edema is a common clinical problem and the etiology is usually clear: cirrhosis, nephrotic syndrome, primary gastrointestinal disorders, malnutrition, etc. We present a 23-year-old previously healthy woman of Korean background who presented with generalized edema and a serum albumin of 9 g/L (normal, 35-45 g/L). Intensive investigations failed to reveal liver, renal, or inflammatory gastrointestinal mucosal disease. The antinuclear antibody was positive at a titer of 1:80, and extractable nuclear antigens were positive for SSA/anti-Ro. Anti-double-stranded DNA was markedly elevated at 4.6 kU/L (normal, 0-2.0 kU/L). A technetium 99M-labeled albumin study revealed a protein-losing enteropathy, despite normal histologic full-thickness jejunal biopsies. A diagnosis of occult systemic lupus erythematosus resulting in increased intestinal vascular permeability was made. The hypoalbuminemia remained in long-term remission after the initiation of induction and maintenance immunosuppression.