Kerin K, Yost J H
Dartmouth-Hitchcock Medical Center, Lebanon, NH, USA.
Compr Ther. 1998 Nov-Dec;24(11-12):574-81.
Vascular disease is observed in virtually all patients with systemic sclerosis and presents several challenges for the clinician. The alterations in vascular tone noted with Raynaud's phenomenon may have corollaries in both the pathogenesis and management of systemic sclerosis-related internal organ complications. Early recognition of isolated pulmonary hypertension, coronary microcirculatory disease, and scleroderma renal involvement may be the keys to successful outcomes. Future trends in the management of systemic sclerosis may be directed at preventing the development of these vascular syndromes. This may occur through combined therapies directed at the abnormal immune response, disregulated fibroblast function, and resultant abnormal vascular response.
几乎所有系统性硬化症患者都存在血管疾病，这给临床医生带来了诸多挑战。雷诺现象中所观察到的血管张力改变，可能在系统性硬化症相关内脏器官并发症的发病机制和治疗方面都有相应影响。早期识别孤立性肺动脉高压、冠状动脉微循环疾病和硬皮病肾受累情况，可能是取得成功治疗结果的关键。系统性硬化症治疗的未来趋势可能是针对预防这些血管综合征的发生。这可能通过针对异常免疫反应、失调的成纤维细胞功能以及由此产生的异常血管反应的联合治疗来实现。
