The systemic capillary leak syndrome: a case series of 28 patients from a European registry.

The systemic capillary leak syndrome (SCLS) is a rare disease characterized by life-threatening attacks of capillary hyperpermeability. To describe the clinical characteristics, laboratory findings, treatments, and outcomes of patients with SCLS who were not previously reported in the literature. Case series. Patients referred to a European multicenter SCLS registry between January 1997 and July 2010. 28 patients with SCLS. Frequency, severity of attacks, and vital status were assessed every 6 months, from diagnosis to the end of the study. 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. The benefits of preventive treatment could not be precisely ascertained because of the small sample size and because most patients received several treatments. 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. Université Pierre et Marie Curie, Paris, France.

Gousseff M ，Arnaud L ，Lambert M ，Hot A ，Hamidou M ，Duhaut P ，Papo T ，Soubrier M ，Ruivard M ，Malizia G ，Tieulié N ，Rivière S ，Ninet J ，Hatron PY ，Amoura Z ，Capillary Leak Syndrome Registry ... -  《-》

Intravenous immunoglobulin in systemic capillary leak syndrome: a case report and review of literature.

Marra AM ，Gigante A ，Rosato E 《-》

Intravenous Immunoglobulins Improve Survival in Monoclonal Gammopathy-Associated Systemic Capillary-Leak Syndrome.

Monoclonal gammopathy-associated systemic capillary-leak syndrome, also known as Clarkson disease, is a rare condition characterized by recurrent life-threatening episodes of capillary hyperpermeability in the context of a monoclonal gammopathy. This study was conducted to better describe the clinical characteristics, natural history, and long-term outcome of monoclonal gammopathy-associated systemic capillary-leak syndrome. We conducted a cohort analysis of all patients included in the European Clarkson disease (EurêClark) registry between January 1997 and March 2016. From diagnosis to last follow-up, studied outcomes (eg, the frequency and severity of attacks, death, and evolution toward multiple myeloma) and the type of preventive treatments administered were monitored every 6 months. Sixty-nine patients (M/F sex ratio 1:1; mean ± SD age at disease onset 52 ± 12 years) were included in the study. All patients had monoclonal gammopathy of immunoglobulin G type, with kappa light chains in 47 (68%). Median (interquartile range) follow-up duration was 5.1 (2.5-9.7) years. Twenty-four patients (35%) died after 3.3 (0.9-8) years. Fifty-seven (86%) patients received at least one preventive treatment, including intravenous immunoglobulins (IVIg) n = 48 (73.8%), theophylline n = 22 (33.8%), terbutaline n = 22 (33.8%), and thalidomide n = 5 (7.7%). In the 65 patients with follow-up, 5- and 10-year survival rates were 78% (n = 35) and 69% (n = 17), respectively. Multivariate analysis found preventive treatment with IVIg (hazard ratio 0.27; 95% confidence interval, 0.10-0.70; P = .007) and terbutaline (hazard ratio 0.35; 95% confidence interval, 0.13-0.96; P = .041) to be independent predictors of mortality. We describe the largest cohort to date of patients with well-defined monoclonal gammopathy-associated systemic capillary-leak syndrome. Preventive treatment with IVIg was the strongest factor associated with survival, suggesting the use of IVIg as the first line in prevention therapy.

Pineton de Chambrun M ，Gousseff M ，Mauhin W ，Lega JC ，Lambert M ，Rivière S ，Dossier A ，Ruivard M ，Lhote F ，Blaison G ，Alric L ，Agard C ，Saadoun D ，Graveleau J ，Soubrier M ，Lucchini-Lecomte MJ ，Christides C ，Bosseray A ，Levesque H ，Viallard JF ，Tieulie N ，Lovey PY ，Le Moal S ，Bibes B ，Malizia G ，Abgueguen P ，Lifermann F ，Ninet J ，Hatron PY ，Amoura Z ，EurêClark Study Group ... -  《-》

A case report of systemic capillary leak syndrome (Clarkson's disease).

Hollenberg J ，Frykman J ，Lundberg LG ，Forsberg S ... -  《-》

[Periodically leaking capillaries].

Timmers HJ ，Keuter M ，van der Meer JW 《-》