Foti R, Leonardi R, Rondinone R, Di Gangi M, Leonetti C, Canova M, Doria A
Rheumatology Unit, Azienda Ospedaliera Universitaria V. Emanuele, Ferrarotto, S. Bambino Catania, Italy.
Autoimmun Rev. 2008 Feb;7(4):331-9. doi: 10.1016/j.autrev.2007.12.004. Epub 2008 Jan 11.
Fibrosing disorders comprise a wide spectrum of heterogeneous diseases characterized by sclerosis of the dermis, subcutis, and sometimes the underlying soft tissues and bone. The hallmark of this group of diseases is skin thickening as in systemic sclerosis with a different distribution pattern and for this reason they have also been referred to as "scleroderma-like" disorders. These diseases may have a different clinical course ranging from a benign disease with a localized cutaneous involvement, to a widespread, systemic, life-threatening disease. Some of them are associated with autoantibodies and/or autoimmune conditions. An accurate recognition of these scleroderma-like diseases is important for the institution of the most appropriate treatment.
纤维化疾病包括一系列广泛的异质性疾病,其特征为真皮、皮下组织,有时还包括深层软组织和骨骼的硬化。这类疾病的标志是皮肤增厚,如同系统性硬化症那样,但分布模式不同,因此它们也被称为“硬皮病样”疾病。这些疾病的临床病程可能各异,从仅累及局部皮肤的良性疾病,到广泛的、全身性的、危及生命的疾病。其中一些与自身抗体和/或自身免疫性疾病相关。准确识别这些硬皮病样疾病对于采取最恰当的治疗措施至关重要。