Bøe Ole Wilhelm, Sveen Kjell, Børset Magne, Druey Kirk M
Department of Medical Biochemistry, Innlandet Hospital Trust, Lillehammer, Norway.
Department of Cancer Research and Molecular Medicine, Norwegian University of Science and Technology, Trondheim, Norway.
Am J Case Rep. 2018 Feb 16;19:176-182. doi: 10.12659/ajcr.906514.
BACKGROUND Systemic capillary leak syndrome (SCLS) (Clarkson's disease) is a rare disorder of unknown etiology, characterized by transient episodes of hypotension, and the microvascular leak of fluids into the peripheral tissues, resulting in edema. Between 80-90% of patients with SCLS have a concomitant monoclonal gammopathy. Although translational in vitro studies have implicated vascular endothelial barrier dysfunction in the etiology of SCLS, the etiology and disease associations in clinical cases remain unknown. CASE REPORT We report a case of SCLS in a 49-year-old woman who initially presented with an upper respiratory tract infection, which was complicated by edema and compartment syndromes in the extremities that required fasciotomies. Serum levels of the cell surface heparan sulfate proteoglycan, syndecan-1 (CD138), a measure of endothelial surface glycocalyx (ESG) damage, were measured by enzyme-linked immunoassay (ELISA), peaked at up to 500 ng/mL (reference range, 50-100 ng/mL) and normalized on disease remission. CONCLUSIONS This case report supports the view that damage to the microvascular endothelium, has a role in the pathogenesis of acute SCLS. This case also indicated that monitoring serum levels of syndecan-1 (CD138) might be used to monitor the progression and resolution of episodes of SCLS.
背景 系统性毛细血管渗漏综合征（SCLS）（克拉克森病）是一种病因不明的罕见疾病，其特征为短暂性低血压发作以及液体向周围组织的微血管渗漏，从而导致水肿。80%至90%的SCLS患者伴有单克隆丙种球蛋白病。尽管体外转化研究表明血管内皮屏障功能障碍与SCLS的病因有关，但临床病例中的病因及疾病关联仍不明确。病例报告 我们报告一例49岁女性的SCLS病例，该患者最初表现为上呼吸道感染，并发四肢水肿和骨筋膜室综合征，需要进行筋膜切开术。通过酶联免疫吸附测定（ELISA）测量细胞表面硫酸乙酰肝素蛋白聚糖（syndecan-1，CD138）的血清水平，这是内皮表面糖萼（ESG）损伤的一项指标，其峰值高达500 ng/mL（参考范围为50 - 100 ng/mL），且在疾病缓解时恢复正常。结论 本病例报告支持微血管内皮损伤在急性SCLS发病机制中起作用这一观点。该病例还表明，监测syndecan-1（CD138）的血清水平可能用于监测SCLS发作的进展和缓解情况。
