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 gastroint estinal disorders, malnutrition, etc. We present a 23-year-old previously h ealthy woman of Korean background who presented with generalized edema and a serum albumin of 9 g/L (normal, 35-45g/L). Intensive investigations faile d 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 wa s markedly elevated at 4.6 kU/L (normal, 0-2.0 kU/L). A technetium 99M-labe led albumin study revealed a protein-losing enteropathy, despite normal his tologic full-thickness jejunal biopsies. A diagnosis of occult systemic lup us erythematosus resulting in increased intestinal vascular permeability wa s made. The hypoalbuminemia remained in long-term remission after the initi ation of induction and maintenance immunosuppression.