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Review

Recognition and management of idiopathic systemic capillary leak syndrome: an evidence-based review

ORCID Icon, , ORCID Icon &
Pages 331-340 | Received 11 Dec 2017, Accepted 21 Mar 2018, Published online: 02 Apr 2018

References

  • Takabatake T. Systemic capillary leak syndrome. Intern Med. 2002;41(11):909–910.
  • Eo TS, Chun KJ, Hong SJ, et al. Clinical presentation, management, and prognostic factors of idiopathic systemic capillary leak syndrome: a systematic review. J Allergy Clin Immunol Pract. 2017. DOI:10.1016/j.jaip.2017.07.021.
  • Druey KM, Parikh SM. Idiopathic systemic capillary leak syndrome (Clarkson disease). J Allergy Clin Immunol. 2017;140(3):663–670.
  • Chihara R, Nakamoto H, Arima H, et al. Systemic capillary leak syndrome. Intern Med. 2002;41(11):953–956.
  • Dellinger RP, Levy MM, Rhodes A, et al. Surviving sepsis campaign: international guidelines for management of severe sepsis and septic shock: 2012. Crit Care Med. 2013;41(2):580–637.
  • Druey KM, Greipp PR. Narrative review: the systemic capillary leak syndrome. Ann Intern Med. 2010;153(2):90–98.
  • Valderrey FP, Putze GB, Azario JM, et al. Systemic capillary leak syndrome associated with rhabdomyolysis and compartment syndrome. Am J Emerg Med. 1999 Nov 30;17(7):743–744.
  • Tahirkheli NK, Greipp PR. Treatment of the systemic capillary leak syndrome with terbutaline and theophylline: a case series. Ann Intern Med. 1999;130(11):905–909.
  • Kapoor P, Greipp PT, Schaefer EW, et al. Idiopathic systemic capillary leak syndrome (Clarkson’s disease): the Mayo clinic experience. Mayo Clin Proc. 2010;85(10):905–912.
  • Dhir V, Arya V, Chandra Malav I, et al. Idiopathic systemic capillary leak syndrome (SCLS): case report and systematic review of cases reported in the last 16 years. Intern Med. 2007;46(12):899–904.
  • Sion-Sarid R, Lerman-Sagie T, Blumkin L, et al. Neurologic involvement in a child with systemic capillary leak syndrome. Pediatrics. 2010;125(3):e687–92.
  • Hsu P, Xie Z, Frith K, et al. Idiopathic systemic capillary leak syndrome in children. Pediatrics. 2015;135(3):e730–e735.
  • Kerketta J, Lodh M, Mandal K. Clarkson disease—systemic capillary leak syndrome in a 6-year-old girl: case report. Paediatr Int Child Health. 2014;35(2):160–163.
  • Onal H, Aktuglu-Zeybek C, Altun G, et al. Capillary leak syndrome in a 5-month-old infant associated with intractable diarrhoea. Ann Trop Paediatr. 2007;27(1):81–86.
  • Kreimeier U. Pathophysiology of fluid imbalance. Crit Care. 2000;4(Suppl 2):S3.
  • Sousa A, Len O, Escolà-Vergé L, et al. Influenza A virus infection is associated with systemic capillary leak syndrome: case report and systematic review of the literature. Antivir Ther. 2016;21(2):181–183.
  • Perme T, Pokorn M, Markelj G, et al. Two episodes of systemic capillary leak syndrome in an 8-year-old boy, following influenza A virus infection. Pediatr Infect Dis J. 2014;33(2):222–224.
  • Jeong EK, Kim YK, Kim SH, et al. Systemic capillary leak syndrome under general anesthesia: a case report. Korean J Anesthesiol. 2014;66(6):462–466.
  • De Pas T, Curigliano G, Franceschelli L, et al. Gemcitabine-induced systemic capillary leak syndrome. Ann Oncol. 2001;12(11):1651–1652.
  • Zhang F, Yang J, Li Z. Trastuzumab-induced systemic capillary leak syndrome in a breast cancer patient. Pathol Oncol Res. 2014;20(2):435–437.
  • Deeren DH, Zachee P, Malbrain ML. Granulocyte colony-stimulating factor-induced capillary leak syndrome confirmed by extravascular lung water measurements. Ann Hematol. 2005;84(2):89–94.
  • Xie Z, Ghosh CC, Patel R, et al. Vascular endothelial hyperpermeability induces the clinical symptoms of Clarkson disease (the systemic capillary leak syndrome). Blood. 2012;119(18):4321–4332.
  • Clarkson B, Thompson D, Horwith M, et al. Cyclical edema and shock due to increased capillary permeability. Am J Med. 1960;29(2):193–216.
  • Senger DR, Galli SJ, Dvorak AM, et al. Tumor cells secrete a vascular permeability factor that promotes accumulation of ascites fluid. Science. 1983;219(4587):983–985.
  • Sek AC, Xie Z, Terai K, et al. Endothelial expression of endothelin receptor A in the systemic capillary leak syndrome. PLoS ONE. 2015;10(7):e0133266.
  • Stirling CM, Boulton-Jones JM, Simpson K. Progressive oedema in a 30-year-old. Lancet. 1998;352(9126):450.
  • Airaghi L, Montori D, Santambrogio L, et al. Chronic systemic capillary leak syndrome. Report of a case and review of the literature. J Intern Med. 2000;247(6):731–735.
  • Xie Z, Ghosh CC, Parikh SM, et al. Mechanistic classification of the systemic capillary leak syndrome: Clarkson disease. Am J Respir Crit Care Med. 2014;189(9):1145–1147.
  • Nagao Y, Harada H, Yamanaka H, et al. Possible mediators for systemic capillary leak syndrome. Am J Med. 2011;124(1):e7–9.
  • Lesterhuis WJ, Rennings AJ, Leenders WP, et al. Vascular endothelial growth factor in systemic capillary leak syndrome. Am J Med. 2009;122(6):e5–7.
  • Johansson BR, Löfdahl CG. Ultrastructure of the microvessels in skeletal muscle in a case of systemic capillary leak syndrome. Acta Med Scand. 1979;206(1–6:413–416.
  • Assaly R, Olson D, Hammersley J, et al. Initial evidence of endothelial cell apoptosis as a mechanism of systemic capillary leak syndrome. Chest. 2001;120(4):1301–1308.
  • van Hinsbergh VW, van Nieuw Amerongen GP. Intracellular signalling involved in modulating human endothelial barrier function. J Anat. 2002;200(6):549–560.
  • Dowden AM, Rullo OJ, Aziz N, et al. Idiopathic systemic capillary leak syndrome: novel therapy for acute attacks. J Allergy Clin Immunol. 2009;124(5):1111–1113.
  • Cicardi M, Gardinali M, Bisiani G, et al. The systemic capillary leak syndrome: appearance of interleukin-2-receptor-positive cells during attacks. Ann Intern Med. 1990;113(6):475–477.
  • Fardet L, Kerob D, Rybojad M, et al. Idiopathic systemic capillary leak syndrome: cutaneous involvement can be misleading. Dermatology. 2004;209(4):291–295.
  • Navarro C, Garcia-Bragado F, Lima J, et al. Muscle biopsy findings in systemic capillary leak syndrome. Hum Pathol. 1990;21(3):297–301.
  • Amoura Z, Papo T, Ninet J, et al. Systemic capillary leak syndrome: report on 13 patients with special focus on course and treatment. Am J Med. 1997;103(6):514–519.
  • Rizzi R, Curci P, Rina Ldi E, et al. Schnitzler’s syndrome: monoclonal gammopathy associated with chronic urticaria. Acta Haematol. 2008;120(1):1–4.
  • Zhang W, Ewan PW, Lachmann PJ. The paraproteins in systemic capillary leak syndrome. Clin Exp Immunol. 1993;93(3):424–429.
  • Cugno M, Castelli R, Cicardi M. Angioedema due to acquired C1-inhibitor deficiency: a bridging condition between autoimmunity and lymphoproliferation. Autoimmun Rev. 2008;8(2):156–159.
  • Barnadas MA, Cisteró A, Sitjas D, et al. Systemic capillary leak syndrome. J Am Acad Dermatol. 1995;32(2):364–366.
  • Duron L, Delestre F, Amoura Z, et al. [Idiopathic and secondary capillary leak syndromes: A systematic review of the literature]. Rev Med Interne. 2015;36(6):386–394.
  • Günes AR, Berlit P, Weber R. Severe cerebral involvement due to idiopathic systemic capillary leak syndrome. Clin Case Rep. 2016;4(4):429–431.
  • de Chambrun MP, Luyt CE, Beloncle F, et al. The clinical picture of severe systemic capillary-leak syndrome episodes requiring ICU admission. Crit Care Med. 2017;45(7):1216–1223.
  • Kawabe S, Saeki T, Yamazaki H, et al. Systemic capillary leak syndrome. Intern Med. 2002;41(3):211–215.
  • Gousseff M, Amoura Z. [Idiopathic capillary leak syndrome]. Rev Med Interne. 2009;30(9):754–768.
  • Dispenzieri A, Moreno-Aspitia A, Suarez GA, et al. Peripheral blood stem cell transplantation in 16 patients with POEMS syndrome, and a review of the literature. Blood. 2004;104(10):3400–3407.
  • Dybowski F, Sepp N, Bergerhausen HJ, et al. Successful use of anakinra to treat refractory Schnitzler’s syndrome. Clin Exp Rheumatol. 2008;26(2):354–357.
  • Koga H, Tokunaga Y, Hisamoto T, et al. Ratio of serum vascular endothelial growth factor to platelet count correlates with disease activity in a patient with POEMS syndrome. Eur J Intern Med. 2002;13(1):70–74.
  • Saugel B, Umgelter A, Martin F, et al. Systemic capillary leak syndrome associated with hypovolemic shock and compartment syndrome. Use of transpulmonary thermodilution technique for volume management. Scand J Trauma Resusc Emerg Med. 2010;18(1):38.
  • Lee YS, Kim SY, Kwon CW, et al. Two cases of systemic capillary leak syndrome that were treated with pentastarch. Korean J Intern Med. 2007;22(2):130–132.
  • Cooperman LH. Succinylcholine-induced hyperkalemia in neuromuscular disease. JAMA. 1970;213(11):1867–1871.
  • Magorian T, Flannery KB, Miller RD. Comparison of rocuronium, succinylcholine, and vecuronium for rapid-sequence induction of anesthesia in adult patients. Anesthesiology. 1993;79(5):913–918.
  • Muzi M, Berens RA, Kampine JP, et al. Venodilation contributes to propofol-mediated hypotension in humans. Anesth Analg. 1992;74(6):877–883.
  • Murray H, Marik PE. Etomidate for endotracheal intubation in sepsis: acknowledging the good while accepting the bad. Chest. 2005;127(3):707–709.
  • Koul RL, Aithala GR, Chacko A, et al. Continuous midazolam infusion as treatment of status epilepticus. Arch Dis Child. 1997;76(5):445–448.
  • Hermens JM, Ebertz JM, Hanifin JM, et al. Comparison of histamine release in human skin mast cells induced by morphine, fentanyl, and oxymorphone. Anesthesiology. 1985;62(2):124–129.
  • Waller JL, Cc H Jr, Nagle DM, et al. Hemodynamic changes during fentanyl–oxygen anesthesia for aortocoronary bypass operation. Anesthesiology. 1981;55(3):212–217.
  • Umbrello M, Gardinali M, Ottolina D, et al. Systemic capillary leak syndrome: is methylene blue the silver bullet? Case Rep Crit Care. 2014;2014:141670.
  • Sheehan JR, Keating L, Chan A, et al. Distributive shock due to systemic capillary leak syndrome treated with high-dose immunosuppression. BMJ Case Rep. 2013;2013:bcr2013009048.
  • Scanvion Q, Lefèvre G, Hachulla E, et al. Subcutaneous immunoglobulin therapy prevents systemic capillary leak syndrome attack. Am J Med. 2016;129(7):e77–8.
  • Lassoued K, Clauvel JP, Similowski T, et al. Pulmonary infections associated with systemic capillary leak syndrome attacks in a patient with hypogammaglobulinemia. Intensive Care Med. 1998;24(9):981–983.
  • Marra AM, Gigante A, Rosato E. Intravenous immunoglobulin in systemic capillary leak syndrome: a case report and review of literature. Expert Rev Clin Immunol. 2014;10(3):349–352.
  • Bayary J, Dasgupta S, Misra N, et al. Intravenous immunoglobulin in autoimmune disorders: an insight into the immunoregulatory mechanisms. Int Immunopharmacol. 2006;6(4):528–534.
  • Gousseff 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(7):464–471.
  • Lambert M, Launay D, Hachulla E, et al. High-dose intravenous immunoglobulins dramatically reverse systemic capillary leak syndrome. Crit Care Med. 2008;36(7):2184–2187.
  • Xie Z, Chan EC, Long LM, et al. High-dose intravenous immunoglobulin therapy for systemic capillary leak syndrome (Clarkson disease). Am J Med. 2015;128(1):91–95.
  • del Olmo RM, Dá A, Santos PM. [Use of intravenous immunoglobulins in a patient with systemic capillary leak syndrome]. Med Clin (Barc). 2014;142(8):377–378.
  • Almagro P, Marti JM, Pascual LG, et al. Successful treatment of systemic capillary leak syndrome with intravenous immunoglobulins. Rev Clin Esp. 2012;212(4):218–219.
  • Zipponi M, Eugster R, Birrenbach T. High-dose intravenous immunoglobulins: a promising therapeutic approach for idiopathic systemic capillary leak syndrome. BMJ Case Rep. 2011;2011:bcr1220103599.
  • Abgueguen P, Chennebault JM, Pichard E. Immunoglobulins for treatment of systemic capillary leak syndrome. Am J Med. 2010;123(6):e3–4.
  • Shin JI, Lee JS. Beneficial effect of intravenous immunoglobulins on systemic capillary leak syndrome in patients with monoclonal gammopathy. Crit Care Med. 2009;37(2):795.
  • de Chambrun MP, Gousseff M, Mauhin W, et al. Intravenous immunoglobulins improve survival in monoclonal gammopathy-associated systemic capillary leak-syndrome. Am J Med. 2017;130(10):1219.e19–27.
  • Droder RM, Kyle RA, Greipp PR. Control of systemic capillary leak syndrome with aminophylline and terbutaline. Am J Med. 1992;92(5):523–526.
  • Keller KK, Hauge EM, Stengaard-Pedersen K. Chronic systemic capillary leak syndrome: a case responding to prednisolone treatment. Scand J Rheumatol. 2009;38(5):400–401.
  • Rondeau E, Sraer J, Bens M, et al. Production of 5‐lipoxygenase pathway metabolites by peripheral leucocytes in capillary leak syndrome (Clarkson disease). Eur J Clin Invest. 1987;17(1):53–57.
  • Wålinder O, Einarsson P, Lindberger K. [A case report of systemic capillary leak syndrome. Effective treatment with immunoadsorption and leukotriene antagonist]. Lakartidningen. 2004;101(38):2880–2882.
  • Staak JO, Glossmann JP, Esser JM, et al. Thalidomide for systemic capillary leak syndrome. Am J Med. 2003;115(4):332–334.
  • Fellows IW, Powell RJ, Toghill PJ, et al. Epoprostenol in systemic capillary leak syndrome. Lancet. 1988;332(8620):1143.
  • Beermann W, Hörstrup KA, Will R, et al. Systemic capillary leak syndrome. Am J Med. 1998;105(6):554.
  • Vigneau C, Haymann JP, Khoury N, et al. An unusual evolution of the systemic capillary leak syndrome. Nephrol Dial Transplant. 2002;17(3):492–494.

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