Intravenous Immunoglobulins Improve Survival in Monoclonal Gammopathy-Associated Systemic Capillary-Leak Syndrome.
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Pineton de Chambrun Marc, Gousseff Marie, Mauhin Wladimir, Lega Jean-Christophe, Lambert Marc, Rivière Sophie, Dossier Antoine, Ruivard Marc, Lhote François, Blaison Gilles, Alric Laurent, Agard Christian, Saadoun David, Graveleau Julie, Soubrier Martin, Lucchini-Lecomte Marie-Josée, Christides Christine, Bosseray Annick, Levesque Hervé, Viallard Jean-François, Tieulie Nathalie, Lovey Pierre-Yves, Le Moal Sylvie, Bibes Béatrice, Malizia Giuseppe, Abgueguen Pierre, Lifermann François, Ninet Jacques, Hatron Pierre-Yves, Amoura Zahir, EurêClark Study Group
Аннотация
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.