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

Autor:	Pineton de Chambrun M; Service de médecine interne 2, CHU La Pitié-Salpêtrière, APHP, Université Paris 6, France; Service de réanimation médicale, CHU La Pitié-Salpêtrière, APHP, Université Paris 6, France., Gousseff M; Service de médecine interne, CH Bretagne Atlantique, Vannes, France., Mauhin W; Service de médecine interne 2, CHU La Pitié-Salpêtrière, APHP, Université Paris 6, France., Lega JC; Service de médecine interne et vasculaire, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, CHU, Pierre-Bénite, France and UMR 5558, Laboratoire de Biométrie et Biologie Évolutive, CNRS, Université Claude-Bernard Lyon 1, France., Lambert M; Service de médecine interne, Hôpital Claude Huriez, CHRU Lille, France., Rivière S; Service de médecine interne, Hôpital Saint-Eloi, CHRU Montpellier, France., Dossier A; Service de médecine interne, CHU Bichat, Paris, APHP, France., Ruivard M; Service de médecine interne, Hôpital d'Estaing, CHU de Clermont-Ferrand, France., Lhote F; Service de médecine interne, Hôpital Delafontaine, Saint-Denis, France., Blaison G; Service de médecine interne et rhumatologie, Hôpital Pasteur, Colmar, France., Alric L; Service de médecine interne, Pôle digestif, CHU Purpan, Toulouse, France., Agard C; Service de médecine interne, CHU Hôtel-Dieu, Nantes, France., Saadoun D; Service de médecine interne et immunologie clinique, CHU La Pitié-Salpêtrière, APHP, Paris, France., Graveleau J; Service de médecine polyvalente, CH Saint-Nazaire, France., Soubrier M; Service de rhumatologie, Hôpital Gabriel-Montpied, CHU Clermont-Ferrand, France., Lucchini-Lecomte MJ; Service de médecine Interne, CH Notre-Dame de la Miséricorde, Ajaccio, France., Christides C; Service de médecine interne, CH Avignon, France., Bosseray A; Service de médecine interne, CHU Grenoble, France., Levesque H; Normandie univ, UNIROUEN, U1096, Service de médecine interne, Rouen, France., Viallard JF; Service de médecine interne, Hôpital Haut-Lévêque, CHU Bordeaux Sud, Pessac, France., Tieulie N; Service de rhumatologie, Hôpital Pasteur 2, CHU Nice, France., Lovey PY; Service d'hématologie, Hôpital du Valais-Institut Central, Sion, Switzerland., Le Moal S; Service de médecine interne, CH de Saint-Brieuc, France., Bibes B; Service de médecine interne, CHP Saint-Grégoire, France., Malizia G; Divisione di Gastroenterologia, Ospedale V. Cervello, Palermo, Italy., Abgueguen P; Service des maladies infectieuses et tropicales, CHU d'Angers, France., Lifermann F; Service de médecine interne, CH Dax, France., Ninet J; Service de médecine interne, CHU Edouard Herriot, Lyon, France., Hatron PY; Service de médecine interne, Hôpital Claude Huriez, CHRU Lille, France., Amoura Z; Service de médecine interne 2, CHU La Pitié-Salpêtrière, APHP, Université Paris 6, France. Electronic address: zahir.amoura@aphp.fr.
Jazyk:	angličtina
Zdroj:	The American journal of medicine [Am J Med] 2017 Oct; Vol. 130 (10), pp. 1219.e19-1219.e27. Date of Electronic Publication: 2017 Jun 09.
DOI:	10.1016/j.amjmed.2017.05.023
Abstrakt:	Background: 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. 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. (Copyright © 2017 Elsevier Inc. All rights reserved.)
Databáze:	MEDLINE
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