A Case of Systemic Capillary Leak Syndrome Following Upper Respiratory Tract Infection / 대한신장학회잡지

ABSTRACT

Systemic capillary leak syndrome is a rare condition characterized by recurrent episodes of generalized edema and severe hypotension associated with paraproteinaemia. We experienced a case of recurrent systemic capillary leak syndrome. A 30-year-old woman had flu-like syndrome with fever, chill, and sore throat. Two days later, she presented progressive generalized edema and weight gain of 4 kg. Biological examination showed hemoconcentration, leukocytosis, and hypoalbuminemia without proteinuria. A radiograph of the chest showed pleural effusion. She had no evidence of heart failure, renal failure, or a protein losing enteropathy, and no abnormalities of liver function. Anti-neuclear antibody and antibodies to ds-DNA were negative. C3 and C4 were decreased. Serum protein electrophoresis showed oligoclonal banding consisting of IgG bands. Bone marrow biopsy was normal and there was no Bence-Jones protein in urine. She experienced a progressive reduction in her weight and a resolution of the peripheral edema without specific treatment. Five months later, she was readmitted with severe episode of shock. After a total of 10 L of fluid was administered intravenously, pulmonary edema developed. Intravenous steroid and theophylline were given which resulted in clinical improvement, accompanied by diuresis. Oral theophylline has been administered for prevention of this crisis up to the present time.