Effective secondary prevention of idiopathic systemic capillary leakage syndrome with intravenous immunoglobulin: a systematic review_Chinese Journal of Evidence-Based Medicine

Authors：

LI Yan , ZHANG Xinyi , TANG Wenjiao ,  ZHANG Li , PAN Ling , NIU Ting

Department of Hematology, West China Hospital, Sichuan University, Chengdu 610041, P. R. China;

Corresponding author：

ZHANG Li, Email: drzhangli2014@sina.com

Keywords：

Systemic capillary leak syndrome; Clarkson disease; Intravenous immunoglobulin; Prophylactic treatment; Monoclonal immunoglobulin

DOI：

10.7507/1672-2531.202208046

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Objective To evaluate the effect of intravenous immunoglobulin (IVIG) on prognosis of patients with idiopathic systemic capillary leakage syndrome (ISCLS). Methods Case reports and case series related to IVIG on prognosis of ISCLS were electronically searched from the PubMed, CNKI and WanFang Data databases from inception to December 31, 2021. Two researchers screened literature and extracted the data independently, then, prognostic data were analyzed. Results A total of 143 case reports (175 patients) and 5 case series (169 patients) were included. About 75% of patients had monoclonal gamma globulin, most of those were IgG κ type. A total of 40 patients received prophylaxis with IVIG, most of whom received a high dose (2 g/kg) of IVIG per month. The 5-year and 10-year survival rates of ISCLS patients receiving IVIG secondary prevention treatment were 96% and 72%, respectively, significantly better than the rates of 66% and 43% in the group without IVIG. The median number of acute episodes per year was 0 (0-20) in the group receiving secondary prevention with IVIG and 2 (1-16) in the group not receiving IVIG. Conclusion High-dose (2g/kg) IVIG can improve the long-term survival of ISCLS patients, but efficacy of IVIG in acute episodes is unclear.

Citation： LI Yan, ZHANG Xinyi, TANG Wenjiao, ZHANG Li, PAN Ling, NIU Ting. Effective secondary prevention of idiopathic systemic capillary leakage syndrome with intravenous immunoglobulin: a systematic review. Chinese Journal of Evidence-Based Medicine, 2023, 23(7): 797-801. doi: 10.7507/1672-2531.202208046 Copy

1.	Clarkson B, Thompson D, Horwith M, et al. Cyclical edema and shock due to increased capillary permeability. Am J Med, 1960, 29: 193-216.
2.	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.
3.	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.
4.	Druey KM, Greipp PR. Narrative review: the systemic capillary leak syndrome. Ann Intern Med, 2010, 153(2): 90-98.
5.	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.
6.	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.
7.	Pineton de Chambrun M, 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.e19-1219.e27.
8.	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, 2018, 6(2): 609-618.
9.	Druey KM, Parikh SM. Idiopathic systemic capillary leak syndrome (Clarkson disease). J Allergy Clin Immunol, 2017, 140(3): 663-670.
10.	Xie Z, Chan E, Yin Y, et al. Inflammatory markers of the systemic capillary leak syndrome (Clarkson disease). J Clin Cell Immunol, 2014, 5: 1000213.
11.	Wu MA, Colombo R, Podda GM, et al. Handling shock in idiopathic systemic capillary leak syndrome (Clarkson's disease): less is more. Intern Emerg Med, 2019, 14(5): 723-730.
12.	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.
13.	Pineton de Chambrun M, 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.
14.	Alkhunaizi AM, Kabbani AH, ElTigani MA. Chronic idiopathic systemic capillary leak syndrome: a case report. Allergy Asthma Clin Immunol, 2019, 15: 34.
15.	Ibata S, Sato T, Takada K, et al. Isoform D of vascular endothelial growth factor in systemic capillary leak syndrome: a case report. J Med Case Rep, 2016, 10: 125.
16.	Berti de Marinis G, Bertozzi I, Allemand E, et al. A 40-year-old man with recurrent fainting, hypotension, lower limb edema and oliguria with body weight gain and secondary erythrocytosis. Intern Emerg Med, 2012, 7(5): 453-456.
17.	Almagro P, Martí JM, Garcia Pascual L, et al. Successful treatment of systemic capillary leak syndrome with intravenous immunoglobulins. Rev Clin Esp, 2012, 212(4): 218-219.
18.	Pecker M, Adams M, Graham W. The systemic capillary leak syndrome. Ann Intern Med, 2011, 155(5): 335-336.
19.	Staak JO, Glossmann JP, Esser JM, et al. Thalidomide for systemic capillary leak syndrome. Am J Med, 2003, 115(4): 332-334.
20.	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.
21.	Cheung PC, Eisch AR, Maleque N, et al. Fatal exacerbations of systemic capillary leak syndrome complicating coronavirus disease. Emerg Infect Dis, 2021, 27(10): 2529-2534.
22.	Matheny M, Maleque N, Channell N, et al. Severe exacerbations of systemic capillary leak syndrome after COVID-19 vaccination: a case series. Ann Intern Med, 2021, 174(10): 1476-1478.
23.	Lesterhuis WJ, Rennings AJ, Leenders WP, et al. Vascular endothelial growth factor in systemic capillary leak syndrome. Am J Med, 2009, 122(6): e5-e7.
24.	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.
25.	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.
26.	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.
27.	Bayry 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.

1. Clarkson B, Thompson D, Horwith M, et al. Cyclical edema and shock due to increased capillary permeability. Am J Med, 1960, 29: 193-216.
2. 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.
3. 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.
4. Druey KM, Greipp PR. Narrative review: the systemic capillary leak syndrome. Ann Intern Med, 2010, 153(2): 90-98.
5. 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.
6. 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.
7. Pineton de Chambrun M, 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.e19-1219.e27.
8. 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, 2018, 6(2): 609-618.
9. Druey KM, Parikh SM. Idiopathic systemic capillary leak syndrome (Clarkson disease). J Allergy Clin Immunol, 2017, 140(3): 663-670.
10. Xie Z, Chan E, Yin Y, et al. Inflammatory markers of the systemic capillary leak syndrome (Clarkson disease). J Clin Cell Immunol, 2014, 5: 1000213.
11. Wu MA, Colombo R, Podda GM, et al. Handling shock in idiopathic systemic capillary leak syndrome (Clarkson's disease): less is more. Intern Emerg Med, 2019, 14(5): 723-730.
12. 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.
13. Pineton de Chambrun M, 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.
14. Alkhunaizi AM, Kabbani AH, ElTigani MA. Chronic idiopathic systemic capillary leak syndrome: a case report. Allergy Asthma Clin Immunol, 2019, 15: 34.
15. Ibata S, Sato T, Takada K, et al. Isoform D of vascular endothelial growth factor in systemic capillary leak syndrome: a case report. J Med Case Rep, 2016, 10: 125.
16. Berti de Marinis G, Bertozzi I, Allemand E, et al. A 40-year-old man with recurrent fainting, hypotension, lower limb edema and oliguria with body weight gain and secondary erythrocytosis. Intern Emerg Med, 2012, 7(5): 453-456.
17. Almagro P, Martí JM, Garcia Pascual L, et al. Successful treatment of systemic capillary leak syndrome with intravenous immunoglobulins. Rev Clin Esp, 2012, 212(4): 218-219.
18. Pecker M, Adams M, Graham W. The systemic capillary leak syndrome. Ann Intern Med, 2011, 155(5): 335-336.
19. Staak JO, Glossmann JP, Esser JM, et al. Thalidomide for systemic capillary leak syndrome. Am J Med, 2003, 115(4): 332-334.
20. 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.
21. Cheung PC, Eisch AR, Maleque N, et al. Fatal exacerbations of systemic capillary leak syndrome complicating coronavirus disease. Emerg Infect Dis, 2021, 27(10): 2529-2534.
22. Matheny M, Maleque N, Channell N, et al. Severe exacerbations of systemic capillary leak syndrome after COVID-19 vaccination: a case series. Ann Intern Med, 2021, 174(10): 1476-1478.
23. Lesterhuis WJ, Rennings AJ, Leenders WP, et al. Vascular endothelial growth factor in systemic capillary leak syndrome. Am J Med, 2009, 122(6): e5-e7.
24. 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.
25. 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.
26. 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.
27. Bayry 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.

Chinese Journal of Evidence-Based Medicine

Effective secondary prevention of idiopathic systemic capillary leakage syndrome with intravenous immunoglobulin: a systematic review

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