Tahirkheli N K, Greipp P R
Mayo Clinic and Mayo Foundation, Rochester, Minnesota 55905, USA.
Ann Intern Med. 1999 Jun 1;130(11):905-9. doi: 10.7326/0003-4819-130-11-199906010-00015.
The systemic capillary leak syndrome is a rare idiopathic disorder characterized by recurrent episodes of hypotension and hemoconcentration due to sudden transient extravasation of 10% to 70% of plasma. Mortality rates 5 years after diagnosis have been reported to be 76%.
To assess the efficacy of a prophylactic regimen for the systemic capillary leak syndrome.
Case series.
Tertiary referral center.
Eight patients followed over the past 18 years.
Oral terbutaline plus aminophylline or theophylline.
Long-term clinical follow-up.
During a median follow-up of 9 years (range, 2 to 18 years), two patients (25%) died: one during an acute episode and one of complications related to long-term corticosteroid therapy. The other six patients are alive and healthy. The frequency and severity of the episodes decreased by a median of 30-fold. Recurrences were associated with decreased serum theophylline levels, possibly caused by enzyme induction or autoinduction. The extended-release form of medication was more successful. Sympathomimetic side effects were significant.
A regimen of terbutaline and theophylline seems to be effective prophylaxis against the systemic capillary leak syndrome. Maintenance of therapeutic drug levels was associated with favorable results.
系统性毛细血管渗漏综合征是一种罕见的特发性疾病，其特征为由于10%至70%的血浆突然短暂外渗而反复出现低血压和血液浓缩。据报道，诊断后5年的死亡率为76%。
评估系统性毛细血管渗漏综合征预防性治疗方案的疗效。
病例系列。
三级转诊中心。
过去18年中随访的8例患者。
口服特布他林加氨茶碱或茶碱。
长期临床随访。
在中位随访9年（范围2至18年）期间，2例患者（25%）死亡：1例死于急性发作，1例死于与长期皮质类固醇治疗相关的并发症。其他6例患者存活且健康。发作的频率和严重程度中位数下降了30倍。复发与血清茶碱水平降低有关，可能是由酶诱导或自身诱导引起的。缓释剂型的药物治疗更成功。拟交感神经副作用显著。
特布他林和茶碱方案似乎是预防系统性毛细血管渗漏综合征的有效方法。维持治疗药物水平与良好结果相关。
