Amoura Z, Papo T, Ninet J, Hatron P Y, Guillaumie J, Piette A M, Blétry O, Dequiedt P, Talasczka A, Rondeau E, Dutel J L, Wechsler B, Piette J C
Service de Médecine Interne, Hôpital de la Pitié, Paris, France.
Am J Med. 1997 Dec;103(6):514-9. doi: 10.1016/s0002-9343(97)00272-6.
Systemic capillary leak syndrome (SCLS) is a rare condition characterized by unexplained episodic capillary hyperpermeability due to a shift of fluid and protein from the intravascular to the interstitial space. This results in diffuse swelling, weight gain, and renal shut-down. From the first publication in 1960, only 34 cases have been reported.
To collate enough patients to observe the natural history of the disease and evaluate the efficacy of empiric treatments.
Multicentric retrospective study.
Thirteen patients (6 women and 7 men) were collated with a mean follow-up of 6.4 years. Eight patients are still alive after a mean of 5.6 years (range 1 to 15). Three patients out of the 11 who were not lost to follow-up died; 1 during an attack and 2 because of a progression towards multiple myeloma.
Our series shows an improvement in the prognosis of SCLS due most likely to improved management during attacks. Some patients' disease could evolve into a multiple myeloma. Treatment is still empiric and no prophylactic therapy, including terbutaline associated with aminophylline, has clearly proven its efficacy.
系统性毛细血管渗漏综合征（SCLS）是一种罕见病症，其特征为不明原因的间歇性毛细血管通透性增加，这是由于液体和蛋白质从血管内转移至间质间隙所致。这会导致全身肿胀、体重增加及肾衰竭。自1960年首次发表相关报道以来，仅报告了34例病例。
收集足够数量的患者，以观察该疾病的自然病程并评估经验性治疗的疗效。
多中心回顾性研究。
共收集了13例患者（6名女性和7名男性），平均随访时间为6.4年。平均5.6年（范围1至15年）后，8例患者仍存活。在11例未失访的患者中，3例死亡；1例死于发作期，2例因病情进展为多发性骨髓瘤死亡。
我们的系列研究表明，SCLS的预后有所改善，这很可能是由于发作期管理的改善。部分患者的疾病可能会演变为多发性骨髓瘤。治疗仍为经验性治疗，包括特布他林联合氨茶碱在内的预防性治疗均未明确证实其疗效。
