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 β(2)-agonists or intravenous immunoglobulin may reduce the frequency and severity of attacks and may improve survival.

PRIMARY FUNDING SOURCE

Université Pierre et Marie Curie, Paris, France.