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.
Northcott
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KA
Department of Medicine, the University of British Columbia, Vancouver, British Columbia, Canada.
Yoshida
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Steinbrecher
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UP
D000328:Adult; D000974:Antibodies, Antinuclear / Q000032:analysis*; D001379:Azathioprine / Q000008:administration & dosage; D001707:Biopsy, Needle; D004247:DNA / Q000032:analysis*; D004035:Diet Therapy / Q000379:methods; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007583:Jejunum / Q000473:pathology*; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis* / Q000628:therapy; D011241:Prednisone / Q000008:administration & dosage; D011504:Protein-Losing Enteropathies / Q000175:diagnosis* / Q000209:etiology* / Q000628:therapy; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
D000974:Antibodies, Antinuclear; D004247:DNA; D001379:Azathioprine; D011241:Prednisone