Advertisement

High-dose Intravenous Immunoglobulin Therapy for Systemic Capillary Leak Syndrome (Clarkson Disease)

Published:September 02, 2014DOI:https://doi.org/10.1016/j.amjmed.2014.08.015

      Abstract

      Background

      Systemic capillary leak syndrome is a highly rare disorder of unknown cause. The disease is characterized by episodes of transient vascular collapse, which leads to hypotensive shock and anasarca. Previous treatment of this potentially devastating condition has been largely ineffective. We evaluated intravenous immunoglobulin prophylactic therapy in a cohort of 29 patients with systemic capillary leak syndrome in a longitudinal follow-up study.

      Methods

      All patients received treatments at the discretion of their primary providers and retrospectively via questionnaire-recorded symptoms beginning with their first documented episode of systemic capillary leak syndrome to May 31, 2014.

      Results

      A total of 22 of 29 patients responded to the questionnaire, and 18 of the 22 respondents received monthly prophylaxis with intravenous immunoglobulin during the study period for a median interval of 32 months. The median annual attack frequency was 2.6 per patient before intravenous immunoglobulin therapy and 0 per patient after initiation of intravenous immunoglobulin prophylaxis (P = .0001). A total of 15 of 18 subjects with a history of 1 or more acute systemic capillary leak syndrome episodes experienced no further symptoms while taking intravenous immunoglobulin therapy.

      Conclusions

      Intravenous immunoglobulin prophylaxis is associated with a dramatic reduction in the occurrence of systemic capillary leak syndrome attacks in most patients, with minimal side effects. A prospective, randomized trial may be necessary to fully assess the benefits of intravenous immunoglobulin for systemic capillary leak syndrome and to determine the optimal dosage and duration of therapy.

      Keywords

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to The American Journal of Medicine
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Clarkson B.
        • Thompson D.
        • Horwith M.
        • Luckey E.H.
        Cyclical edema and shock due to increased capillary permeability.
        Am J Med. 1960; 29: 193-216
        • Druey K.M.
        • Greipp P.R.
        Narrative review: the systemic capillary leak syndrome.
        Ann Intern Med. 2010; 153: 90-98
        • Kapoor P.
        • Greipp P.T.
        • Schaefer E.W.
        • et al.
        Idiopathic systemic capillary leak syndrome (Clarkson's disease): the Mayo clinic experience.
        Mayo Clin Proc. 2010; 85: 905-912
        • Grousseff M.
        • Arnaud L.
        • Lambert M.
        • et al.
        The systemic capillary leak syndrome: a case series of 28 patients from a European registry.
        Ann Intern Med. 2011; 154: 464-471
        • Tahirkheli N.K.
        • Greipp P.R.
        Treatment of the systemic capillary leak syndrome with terbutaline and theophylline. A case series.
        Ann Intern Med. 1999; 130: 905-909
        • Xie Z.
        • Ghosh C.C.
        • Parikh S.M.
        • Druey K.M.
        Mechanistic classification of the systemic capillary leak syndrome: Clarkson disease.
        Am J Respir Crit Care Med. 2014; 189: 1145-1147
        • Lassoued K.
        • Clauvel J.P.
        • Similowski T.
        • Autran B.
        • Bengoufa D.
        • Oksenhendler E.
        Pulmonary infections associated with systemic capillary leak syndrome attacks in a patient with hypogammaglobulinemia.
        Intensive Care Med. 1998; 24: 981-983
        • Lambert M.
        • Launay D.
        • Hachulla E.
        • et al.
        High-dose intravenous immunoglobulins dramatically reverse systemic capillary leak syndrome.
        Crit Care Med. 2008; 36: 2184-2187
        • Abgueguen P.
        • Chennebault J.M.
        • Pichard E.
        Immunoglobulins for treatment of systemic capillary leak syndrome.
        Am J Med. 2010; 123: e3-e4
        • Govig B.A.
        • Javaheri S.
        The systemic capillary leak syndrome.
        Ann Intern Med. 2010; 153: 764
        • Pecker M.
        • Adams M.
        • Graham W.
        The systemic capillary leak syndrome.
        Ann Intern Med. 2011; 155: 335-336
        • Almagro P.
        • Marti J.M.
        • Garcia Pascual L.
        • Rodriguez-Carballeira M.
        Successful treatment of systemic capillary leak syndrome with intravenous immunoglobulins.
        Rev Clin Esp. 2012; 212: 218-219
        • Rabbolini D.J.
        • Ange N.
        • Walters G.D.
        • Pidcock M.
        • Randall K.L.
        Systemic capillary leak syndrome: recognition prevents morbidity and mortality.
        Int Med J. 2013; 43: 1145-1147
        • Marra A.M.
        • Gigante A.
        • Rosato E.
        Intravenous immunoglobulin in systemic capillary leak syndrome: a case report and review of literature.
        Expert Rev Clin Immunol. 2014; 10: 349-352