Roles of bone marrow-derived cells in pathogenesis of pulmonary arterial hypertension associated with connective tissue diseases

骨髓源性细胞在结缔组织疾病相关肺动脉高压发病机制中的作用

• 批准号: 21390300
• 负责人: KUWANA Masataka
• 金额: $ 11.4万
• 依托单位: Keio University
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Scientific Research (B)
• 财政年份: 2009
• 资助国家: 日本
• 起止时间: 2009 至 2011
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-21390300/
• 关键词: 膠原病学; 肺動脈性肺高血圧症; 強皮症; 血管内皮前駆細胞; 単球; 血管新生; 線維化; 膠原病; 血管リモデリング

Pulmonary arterial hypertension(PAH) associated with systemic sclerosis(SSc) is one of intractable conditions with poor prognosis, but its pathogenic process is largely unknown. We have examined potential roles of endothelial progenitor cells(EPCs), which are involved in vascular repair, in the pathogenesis of PAH associated with SSc. As a result, EPCs in patients with SSc exhibited impaired vascular repair machinery, but had enhanced pro-fibrotic capacity. These EPC properties are the most prominent in SSc patients with PAH, and invasion capacity is augmented in EPCs derived from such patients. In summary, aberrant circulating EPC is a novel therapeutic target in PAH associated with SSc.

合并系统性硬化症（SSc）的肺动脉高压（PAH）是预后不良的难治性疾病之一，但其发病机制尚不清楚。我们研究了参与血管修复的内皮祖细胞 (EPC) 在与 SSc 相关的 PAH 发病机制中的潜在作用。结果，SSc 患者的 EPC 表现出血管修复机制受损，但促纤维化能力增强。这些 EPC 特性在患有 PAH 的 SSc 患者中最为突出，并且源自此类患者的 EPC 的侵袭能力增强。总之，异常循环 EPC 是与 SSc 相关的 PAH 的新治疗靶点。

项目成果

膠原病-新たな治療戦略;膠原病診療の実際:全身性硬化症

胶原病——胶原病治疗新策略实践：系统性硬化症

• 发表时间: 2010
• 期刊: 臨床と研究
• 作者: Lee HM;Sugino H;Aoki C;Shimaoka Y;Suzuki R;Ochi K;Ochi T;Nishimoto N.;桑名正隆
• 通讯作者: 桑名正隆

Organ involvement in early SSc

早期 SSc 的器官受累

• 发表时间: 2011
• 作者: 江里口芳裕;前原依子;下野信行;宮本敏浩;赤司浩一;豊嶋崇徳;Kuwana M
• 通讯作者: Kuwana M

強皮症における循環血管内皮前駆細胞(EPC)数の再検討

硬皮病循环内皮祖细胞 (EPC) 数量的复查

• 发表时间: 2010
• 作者: 岡崎有佳;佐藤隆司;安岡秀剛;竹内勤;桑名正隆
• 通讯作者: 桑名正隆

Mobilization of endothelial progenitor cells by intravenous cyclophosphamide in patients with systemic sclerosis : potential association with efficacy for interstitial lung disease

系统性硬化症患者静脉注射环磷酰胺动员内皮祖细胞：与间质性肺病疗效的潜在关联

• 发表时间: 2010
• 期刊: Rheumatology
• 影响因子: 5.5
• 作者: Furuya Y;Okazaki Y;Kaji K;Sato S;Takehara K;Kuwana M
• 通讯作者: Kuwana M

The gene polymorphism within hepatocyte growth factor promoter controls progression of systemic sclerosis-associated interstitial lung disease.

肝细胞生长因子启动子内的基因多态性控制系统性硬化症相关间质性肺病的进展。

• 发表时间: 2010
• 作者: Satoh T;Hoshino K;Kuwana M
• 通讯作者: Kuwana M