The efficacy of tocilizumab in a patient with pulmonary arterial hypertension associated with Castleman's disease
The efficacy of tocilizumab in a patient with pulmonary arterial hypertension associated with Castleman's disease
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DOI：
10.1007/s00380-009-1215-5
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发表时间：
2010-09-01
影响因子：
1.5
通讯作者：
Yamauchi-Takihara, Keiko
中科院分区：
文献类型：
--
作者：
Arita, Yoh;Sakata, Yasushi;Yamauchi-Takihara, Keiko
Castleman病是一种高度异质性的临床病理实体，属于淋巴组织增生性疾病，在某些患者中与肺动脉高压（PAH）相关。它与白细胞介素-6（IL-6）的过度免疫刺激有关，也参与了PAH的发病机制。1例31岁Castleman病女性患者表现为PAH，特征为重度右心衰竭。由于她对各种常规治疗（包括类固醇、前列环素、波生坦和西地那非）耐药，开始了托珠单抗（抗IL-6受体抗体）治疗。她的临床病程随访6个月，有显著改善，无任何不良反应。这是第一例报告的Castleman病相关PAH患者在类固醇和常规PAH治疗基础上使用托珠单抗的病例。
Castleman's disease is a highly heterogeneous clinical-pathological entity that belongs to the lymphoproliferative disorders and is associated with pulmonary arterial hypertension (PAH) in some patients. It is linked to excessive immune stimulation by interleukin-6 (IL-6), which is also involved in the pathogenesis of PAH. A 31-year-old woman with Castleman's disease demonstrated PAH characterized by severe right heart failure. Since she was resistant to various conventional therapies including steroids, prostacyclins, bosentan, and sildenafil, tocilizumab (anti-IL-6 receptor antibody) therapy was started. Her clinical course was followed for 6 months, with significant improvement without any adverse effect. This is the first reported case of use of tocilizumab in addition to steroids and conventional PAH therapy in a patient with PAH associated with Castleman's disease.