Hinchcliff Monique, Varga John
Northwestern University, Feinberg School of Medicine, Chicago, Illinois 60611, USA.
Am Fam Physician. 2008 Oct 15;78(8):961-8.
Systemic sclerosis (systemic scleroderma) is a chronic connective tissue disease of unknown etiology that causes widespread microvascular damage and excessive deposition of collagen in the skin and internal organs. Raynaud phenomenon and scleroderma (hardening of the skin) are hallmarks of the disease. The typical patient is a young or middle-age woman with a history of Raynaud phenomenon who presents with skin induration and internal organ dysfunction. Clinical evaluation and laboratory testing, along with pulmonary function testing, Doppler echocardiography, and high-resolution computed tomography of the chest, establish the diagnosis and detect visceral involvement. Patients with systemic sclerosis can be classified into two distinct clinical subsets with different patterns of skin and internal organ involvement, autoantibody production, and survival. Prognosis is determined by the degree of internal organ involvement. Although no disease-modifying therapy has been proven effective, complications of systemic sclerosis are treatable, and interventions for organ-specific manifestations have improved substantially. Medications (e.g., calcium channel blockers and angiotensin-II receptor blockers for Raynaud phenomenon, appropriate treatments for gastroesophageal reflux disease) and lifestyle modifications can help prevent complications, such as digital ulcers and Barrett esophagus. Endothelin-1 receptor blockers and phosphodiesterase-5 inhibitors improve pulmonary arterial hypertension. The risk of renal damage from scleroderma renal crisis can be lessened by early detection, prompt initiation of angiotensin-converting enzyme inhibitor therapy, and avoidance of high-dose corticosteroids. Optimal patient care includes an integrated, multidisciplinary approach to promptly and effectively recognize, evaluate, and manage complications and limit end-organ dysfunction.
系统性硬化症（系统性硬皮病）是一种病因不明的慢性结缔组织疾病，可导致广泛的微血管损伤以及皮肤和内脏器官中胶原蛋白过度沉积。雷诺现象和硬皮病（皮肤硬化）是该疾病的标志。典型患者为有雷诺现象病史的年轻或中年女性，表现为皮肤硬结和内脏器官功能障碍。临床评估、实验室检查，以及肺功能测试、多普勒超声心动图和胸部高分辨率计算机断层扫描可确立诊断并检测内脏受累情况。系统性硬化症患者可分为两个不同的临床亚组，其皮肤和内脏器官受累模式、自身抗体产生情况及生存率各不相同。预后取决于内脏器官受累程度。虽然尚未证实有改变病情的疗法有效，但系统性硬化症的并发症是可治疗的，针对器官特异性表现的干预措施也有了显著改善。药物治疗（例如，用于雷诺现象的钙通道阻滞剂和血管紧张素II受体阻滞剂、针对胃食管反流病的适当治疗方法）和生活方式改变有助于预防并发症，如指端溃疡和巴雷特食管。内皮素-1受体阻滞剂和磷酸二酯酶-5抑制剂可改善肺动脉高压。通过早期检测、及时开始使用血管紧张素转换酶抑制剂治疗以及避免使用高剂量糖皮质激素，可以降低硬皮病肾危象导致肾损害的风险。最佳的患者护理包括采用综合、多学科方法，以便迅速有效地识别、评估和管理并发症，并限制终末器官功能障碍。
