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OBJECTIVE: To determine clinical features, natural history, and outcome of a well-defined cohort of 25 consecutive patients with idiopathic systemic capillary leak syndrome (SCLS) evaluated at a tertiary care center. PATIENTS AND METHODS: Records of patients diagnosed as having SCLS from November 1, 1981, through April 30, 2008, were reviewed. Descriptive statistics were used to analyze patient demographics, clinical features, complications, and therapeutic interventions. RESULTS: Of the 34 patients whose records were reviewed, 25 fulfilled all diagnostic criteria for SCLS. The median age at diagnosis of SCLS was 44 years. Median follow-up of surviving patients was 4.9 years, and median time to diagnosis from symptom onset was 1.1 years (Interquartile range, 0.5-4.1 years). Fluilke illness or myaigia was reported by 14 patients (56%) at onset of an acute attack of SCLS, and rhabdomyoiysis developed in 9 patients (36%). Patients with a greater decrease in aibumin level had a higher likehood of developing rhabdomyoiysis (P=.03). Monoclonai gammopathy, predominantly of the lgG-K type, was found. In 19 patients (76%). The progression rate to multiple myeioma was 07% per person-year of follow-up. The overall response rate to the different therapies was 76%, and 24% of patients sustained durable (>2 years) complete remission. The estimated 5-year overaii survival rate was 76% (95% confidence intervai, 59%-97%). CONCLUSION: Systemic capillary leak syndrome, a rare disease that occurs in those of middle age, is usually diagnosed after a considerable delay from onset of symptoms. The degree of albu- min decrement during an attack correlates with development of rhabdomyoiysis. A reduction in the frequency and/or the severity of attacks was seen in nearly three-fourths of patients who were offered empirical therapies. The rate of progression to multiple myeioma appears to be comparable to that of monocional gammopathy of undetermined significance. [ABSTRACT FROM AUTHOR]