The Systemic Capillary Leak Syndrome: A Case Series of 28 Patients From a European Registry

Background: The systemic capillary leak syndrome (SCLS) is a rare disease characterized by life-threatening attacks of capillary hyperpermeability. Objective: To describe the clinical characteristics, laboratory findings, treatments, and outcomes of patients with SCLS who were not previously reported in the literature. Design: Case series. Setting: Patients referred to a European multicenter SCLS registry between January 1997 and July 2010. Patients: 28 patients with SCLS. Measurements: Frequency, severity of attacks, and vital status were assessed every 6 months, from diagnosis to the end of the study. Results: 13 men and 15 women referred to the registry who were not previously reported in the literature had 252 attacks. Median age at disease onset was 49.1 years (range, 5.4 to 77.7 years), and median annual frequency of attacks was 1.23 (range, 0.13 to 21.18) per patient. Monoclonal IgG gammopathy was observed in 25 patients (89%). Preventive treatment included intravenous immunoglobulin (n = 18), terbutaline (n = 9), and aminophylline (n = 10). Eight patients died (29%); 1-year survival was 89%, and 5-year survival was 73%. Death was directly related to SCLS attacks in 6 of 8 cases (75%). In 10 patients with a prediagnosis period greater than 6 months who received preventive treatment, the annual frequency of attacks after diagnosis decreased by a median of 1.55 (range, 0.14 to 8.84) per patient. Five years after diagnosis, survival was 85% in 23 patients who had received prophylactic treatment and 20% in 5 patients who had not. Limitation: The benefits of preventive treatment could not be precisely ascertained because of the small sample size and because most patients received several treatments. Conclusion: Clinical experience with these 28 patients with SCLS suggests that prophylactic treatment with beta(2)-agonists or intravenous immunoglobulin may reduce the frequency and severity of attacks and may improve survival.