Scleroderma - clinical and pathological advances
Scleroderma - clinical and pathological advances
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DOI：
10.1016/j.berh.2004.03.001
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发表时间：
2004-06-01
影响因子：
5.2
通讯作者：
Black, CM
中科院分区：
文献类型：
--
作者：
Denton, CP;Black, CM
硬皮病的范围涵盖雷诺现象、皮肤纤维化的局部形式以及涉及炎症、血管和纤维化病理学的临床上最重要的系统性硬化症形式。现在人们认识到这些不同病症之间的密切关系，并且皮肤硬化不再被视为诊断系统性硬化症的强制性条件。最近，疾病分类、自然史的认识以及器官并发症的调查和治疗方面发生了重大变化。尽管硬皮病的病例特异性死亡率仍然很高，但肾脏和肺部疾病的治疗已取得重大进展，胃肠道受累等领域也往往可以得到改善。对这些领域中的每一个都进行了回顾，并描述了理解发病机制的进展。器官并发症的治疗受益于其他医学分支的进步。用于治疗硬皮病肾危象的血管紧张素转换酶抑制剂、用于治疗反流性食管炎的质子泵抑制剂以及用于 III 类和 IV 类肺动脉高压的先进疗法，体现了系统性硬化症治疗方面的进展。还有一种有针对性的细胞因子导向治疗的前景，这可能首次为早期疾病提供真正的疾病缓解干预的前景。与这些发展同时，疾病评估也取得了实质性进展，用于评估皮肤生物力学、功能损伤以及系统性硬化症的严重性和活动性的工具的构建和初步验证。未来几年进行的临床试验可能会改变系统性硬化症的治疗方法，并有助于消除其作为最难治疗的自身免疫性风湿病之一的声誉。
The spectrum of scleroderma spans Raynaud's phenomenon, localized forms of skin fibrosis and the clinically most important forms of systemic sclerosis that involve inflammatory, vascular and fibrotic pathology. A closer relationship between these disparate conditions is now appreciated, and skin sclerosis is no longer regarded as mandatory for the diagnosis of systemic sclerosis. There have been recent and substantial changes in disease classification, the appreciation of its natural history and the investigation and treatment of organ-based complications. Although scleroderma still has a high case-specific mortality, there have been major improvements in the management of renal and pulmonary disease, and areas such as gastrointestinal tract involvement can also often be improved. Each of these areas is reviewed, and progress in understanding pathogenesisis also described. The management of organ-based complications has benefited from advances in other branches of medicine. Angiotensin-converting enzyme inhibitors for scleroderma renal crisis, proton pump inhibitors for reflux oesophagitis and advanced therapies for classes III and IV pulmonary arterial hypertension exemplify progress in the treatment of systemic sclerosis. There is also the prospect of targeted, cytokine-directed treatments that may for the first time offer the prospect of genuine disease-modifying intervention in early-stage disease. In parallel with these developments, there has been substantial progress in disease assessment with the construction and initial validation of tools to assess skin biomechanics, functional impairment and the severity and activity of systemic sclerosis. It is likely that clinical trials performed over the next few years will transform the management of systemic sclerosis and help to dispel its reputation as one of the least treatable of the autoimmune rheumatic diseases.