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J Allergy Clin Immunol Pract. 2018 Mar - Apr;6(2):609-618. doi: 10.1016/j.jaip.2017.07.021. Epub 2017 Sep 19.

Clinical Presentation, Management, and Prognostic Factors of Idiopathic Systemic Capillary Leak Syndrome: A Systematic Review.

Author information

1
Yonsei University College of Medicine, Seoul, Korea.
2
CHA University School of Medicine, Pocheon, Korea.
3
Chonbuk National University College of Medicine, Jeonju, Korea.
4
Chungnam National University College of Medicine, Daejeon, Korea.
5
Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea; Department of Pediatric Nephrology, Severance Children's Hospital, Seoul, Korea.
6
Department of Paediatrics, Luton & Dunstable University Hospital, NHS Foundation Trust, Luton, United Kingdom.
7
Department of Internal Medicine IV, Medical University Innsbruck, Innsbruck, Austria.
8
Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea; Department of Pediatric Nephrology, Severance Children's Hospital, Seoul, Korea; Institute of Kidney Disease Research, Yonsei University College of Medicine, Seoul, Korea. Electronic address: shinji@yuhs.ac.

Abstract

BACKGROUND:

The idiopathic systemic capillary leak syndrome is a rare disease characterized by unexplained recurrent shock caused by capillary hyperpermeability. Because of the rarity of the disease, this disease has easily been misdiagnosed and evidence of efficacious agents used empirically is lacking.

OBJECTIVE:

To analyze the clinical and laboratory data, treatment modalities, and mortality rate of patients and to find contributing factors leading to mortality.

METHODS:

We searched MEDLINE (inception to December 2016) and reviewed reference lists of previous systematic reviews. A total of 133 case reports (161 patients) and 5 case series (102 patients) of idiopathic systemic capillary leak syndrome were included.

RESULTS:

Patients had hypotension (81.4%), edema (64.6%), and previous flu-like illness (34.2%). This disease was misdiagnosed as hypovolemic shock, septic shock, polycythemia vera, or angioedema. Thirty-seven patients died (23%) mainly because of systemic capillary leak syndrome itself (78.4%). There were significant differences in the survival rates between patients who were treated with prophylactic β2 agonists, methylxanthines, and intravenous immunoglobulins and those who were not. The estimated 1-, 5-, and 10-year survival rate of patients treated with intravenous immunoglobulins was 100%, 94%, and 94%, respectively.

CONCLUSIONS:

We systematically analyzed in detail clinical presentations of all reported patients and identified various factors associated with mortality and effects of prophylactic treatment in idiopathic systemic capillary leak syndrome. The findings of this review will facilitate diagnostic approaches of idiopathic systemic capillary leak syndrome and aid in the selection of treatment.

KEYWORDS:

IVIG; Idiopathic; Intravenous immunoglobulins; Methylxanthines; Mortality; Survival; Systemic capillary leak syndrome; β2 agonists

PMID:
28939140
DOI:
10.1016/j.jaip.2017.07.021

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