Management of Acute Episodes of Clarkson Disease (Monoclonal Gammopathy-Associated Systemic Capillary Leak Syndrome) With Intravenous Immunoglobulins.

Autor:	Pecker MS; Department of Internal Medicine, New York-Presbyterian Hospital/Weill Cornell Medical Center, New York, New York., Hammudi M; Department of Internal Medicine, George Washington University School of Medicine and Health Sciences, Washington, DC., Melchio R; Department of Internal Medicine, Santa Croce e Carle Hospital, Cuneo, Italy., Eisch AR; Lung and Vascular Inflammation Section, Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases/National Institutes of Health, Bethesda, Maryland., Verlicchi F; Transfusion Medicine Faenza-Lugo, Transfusion Service Ravenna, Romagna Health Unit, Ravenna, Italy., Druey KM; Lung and Vascular Inflammation Section, Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases/National Institutes of Health, Bethesda, Maryland.
Jazyk:	angličtina
Zdroj:	Annals of internal medicine. Clinical cases [Ann Intern Med Clin Cases] 2022 Aug; Vol. 1 (6). Date of Electronic Publication: 2022 Aug 02.
DOI:	10.7326/aimcc.2022.0496
Abstrakt:	Monoclonal gammopathy-associated idiopathic systemic capillary leak syndrome (ISCLS, Clarkson disease) is a rare disorder defined by transient but recurrent bouts of hypotensive shock and anasarca resulting from plasma extravasation. Although prophylactic treatment with high-dose intravenous immunoglobulins (IVIG, 1-2 g/kg/mo) prevents most disease flares, its utility for acute episodes of ISCLS is unclear. Here, we report the results of a retrospective study of subjects with acute ISCLS treated at or near the onset of symptoms with IVIG. We found that administration of IVIG with minimal additional intravenous fluids was safe and associated with rapid clinical improvement. IVIG given close to the onset of ISCLS-related symptoms is associated with a favorable outcome.
Databáze:	MEDLINE
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