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首页> 外文期刊>Clinics in dermatology >Scleroderma: Nomenclature, etiology, pathogenesis, prognosis, and treatments: Facts and controversies
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Scleroderma: Nomenclature, etiology, pathogenesis, prognosis, and treatments: Facts and controversies

机译：硬皮病：命名，病因，发病机制，预后和治疗：事实和争议

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摘要

Scleroderma refers to a heterogeneous group of autoimmune fibrosing disorders. The nomenclature of scleroderma has changed dramatically in recent years, with morphea (localized scleroderma), limited cutaneous systemic sclerosis, diffuse cutaneous systemic sclerosis, and systemic sclerosis sine scleroderma encompassing the currently accepted disease subtypes. Major advances have been made in the molecular studies of morphea and systemic sclerosis; however, their etiologies and pathogenesis remain incompletely understood. Although morphea and systemic sclerosis demonstrate activation of similar inflammatory and fibrotic pathways, important differences in signaling pathways and gene signatures indicate they are likely biologically distinct processes. Morphea can cause significant morbidity but does not affect mortality, whereas systemic sclerosis has the highest disease-specific mortality of all autoimmune connective tissue diseases. Treatment recommendations for morphea and systemic sclerosis are based on limited data and largely expert opinions. Current collaborative efforts in morphea and systemic sclerosis research will hopefully lead to better understanding of the etiology and pathogenesis of these rare and varied diseases and improved treatment options.
机译：硬皮病是指一组自身免疫性纤维化疾病。近年来，硬皮病的命名已发生了巨大变化，其中吗啡（局部性硬皮病），有限的皮肤全身性硬化症，弥漫性皮肤全身性硬化症和全身性硬化性正弦硬皮病涵盖了目前公认的疾病亚型。在吗啡和全身性硬化症的分子研究中取得了重大进展。然而，他们的病因和发病机理仍然不完全了解。尽管吗啡和全身性硬化症显示出相似的炎症和纤维化途径的激活，但信号传导途径和基因标记的重要差异表明它们可能是生物学上不同的过程。 Morphea可以引起很大的发病率，但不影响死亡率，而系统性硬化症在所有自身免疫性结缔组织疾病中具有特定疾病的最高死亡率。对吗啡和全身性硬化症的治疗建议是基于有限的数据和很大程度上是专家意见。当前在吗啡和全身性硬化研究中的合作努力有望使人们更好地了解这些罕见和多种疾病的病因和发病机理，并改善治疗选择。

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