特发性系统性毛细血管渗漏综合征10例临床特征分析

Clinical features of idiopathic systemic capillary leakage syndrome: an analysis of 10 cases

目的分析特发性系统性毛细血管渗漏综合征(ISCLS)的临床特征。方法该研究为病例系列分析。回顾性收集1983年1月1日至2023年8月31日北京协和医院住院的ISCLS患者的临床资料, 包括一般情况、症状和体征、实验室检查指标以及治疗和预后等。结果共纳入明确诊断为ISCLS的患者10例, 其中男性8例, 起病年龄29~63岁, ≥40岁者6例。9例患者具有非特异性前驱症状。10例患者渗漏期均出现典型的低血压、血液浓缩、低白蛋白血症以及急性肾损伤, 恢复期血流动力学指标均恢复正常。6例患者合并神经系统异常。渗漏期, 3例患者出现凝血功能异常, 4例患者白细胞介素(IL)-6、IL-8、肿瘤坏死因子-α升高;10例患者的外周血均检测到M蛋白, 8例为免疫球蛋白(Ig)Gκ, 2例为IgGλ。急性期治疗以补液、升压治疗为主;稳定期4例患者接受抗浆细胞治疗后发作减少, 2例患者输注静脉用丙种球蛋白后仍有发作, 2例患者接受茶碱类药物治疗预后不佳, 1例患者未治疗自行好转, 1例患者失访。结论 ISCLS临床罕见, 该医院收治的ISCLS患者均为成年后起病, 前驱症状不具有特异性, 发作时低血压表现突出, 均合并单克隆丙种球蛋白血症, 稳定期尚缺乏有效的治疗方法。

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 IgGκand 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.