Clinical features of idiopathic systemic capillary leakage syndrome: an analysis of 10 cases
10.3760/cma.j.cn114798-20240911-00746
- VernacularTitle:特发性系统性毛细血管渗漏综合征10例临床特征分析
- Author:
Juan WU
1
;
Xinxin HAN
;
Hong DI
;
Yue YIN
;
Yingdong HAN
;
Yun ZHANG
;
Xuejun ZENG
Author Information
1. 中国医学科学院 北京协和医学院 北京协和医院全科医学科（普通内科）疑难重症及罕见病国家重点实验室，北京 100730
- Keywords:
Capillary leak syndrome;
Hypotension;
Monoclonal gammopathy
- From:
Chinese Journal of General Practitioners
2024;23(11):1195-1201
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To analyze the clinical characteristics of idiopathic systemic capillary leak syndrome (ISCLS).Methods:This retrospective case series analysis included 10 patients with confirmed ISCLS who were hospitalized at Peking Union Medical College Hospital from January 1983 to August 2023. Data collected encompassed general information, symptoms, laboratory results, treatment, and prognosis.Results:Among 10 ISCLS patients, 8 were males and 2 were females. The age of onset ranged from 29 to 63 years, and it was ≥40 years in 6 cases. Nine patients had nonspecific prodromal symptoms. All 10 experienced hypotension, hemoconcentration, hypoalbuminemia, and acute kidney injury during the leakage phase, with hemodynamic recovery in the subsequent phase. Neurological abnormalities were observed in 6 patients. Three patients had coagulation disorders, and 4 showed elevated levels of IL-6, IL-8, and TNF-α. M protein was found in all patients, with 8 showing IgGκ and 2 IgGλ. Acute-phase treatment primarily involved fluid resuscitation and vasopressors. In the stable phase, 4 patients had fewer episodes with anti-plasma cell therapy; 2 continued to have episodes despite intravenous immunoglobulin (IVIG); 2 had poor outcomes with theophylline; 1 improved without treatment; and 1 was lost to follow-up.Conclusions:ISCLS is a rare disease with adult-onset, presenting nonspecific prodromal symptoms and marked hypotension during episodes. All patients had monoclonal gammopathy. Effective treatment for the stable phase is lacking, though anti-plasma cell therapy may offer some benefit.
