Yost J H, Spencer-Green G
Dartmouth-Hitchcock Medical Center, Lebanon, NH 03756.
Compr Ther. 1993;19(2):53-9.
Vascular disease is seen in virtually all patients with systemic sclerosis and presents several challenges to physicians caring for them. Early recognition of isolated pulmonary hypertension and scleroderma renal crisis may be keys to successful outcomes. Although complete reversal of vascular disease is usually not possible, the availability of calcium channel blocking agents for RP and isolated pulmonary hypertension and ACE inhibitors for hypertensive renal disease has improved the morbidity and mortality of these patients. Optimal management of the vascular events in systemic sclerosis will ultimately depend on a clearer understanding of their pathogenesis. Treatment may ultimately be directed at preventing the development of these vascular syndromes. This may occur through combined therapy directed at both the abnormal immune response and disregulated fibroblast function seen in the disease, as well at the abnormal vascular responses. Such therapies have yet to be identified.
几乎所有系统性硬化症患者都存在血管疾病，这给照料他们的医生带来了诸多挑战。早期识别孤立性肺动脉高压和硬皮病肾危象可能是取得成功治疗结果的关键。尽管血管疾病通常无法完全逆转，但用于治疗雷诺现象和孤立性肺动脉高压的钙通道阻滞剂以及用于治疗高血压肾病的血管紧张素转换酶抑制剂的出现，已改善了这些患者的发病率和死亡率。系统性硬化症血管病变的最佳管理最终将取决于对其发病机制更清晰的认识。治疗最终可能旨在预防这些血管综合征的发生。这可能通过针对该疾病中出现的异常免疫反应、失调的成纤维细胞功能以及异常血管反应的联合治疗来实现。此类治疗方法尚未确定。
