Walecka Irena
Clinical Department of Dermatology, Central Clinical Hospital of the MSWiA, Warsaw, Poland.
Prz Gastroenterol. 2017;12(3):163-168. doi: 10.5114/pg.2017.70467. Epub 2017 Sep 30.
Systemic sclerosis (SSc) is an autoimmunological disease of unknown origin with complex pathogenesis and multiple organ involvement. It is characterised by vascular and immunological abnormalities leading to fibrosis of the skin and internal organs. It is a rather rare disease with a prevalence of around 20 per 100,000. The disease results in heterogeneous clinical findings and different courses. Systemic sclerosis usually begins with the onset of Raynaud's phenomenon (RP), followed by skin sclerosis and internal organ involvement, although it may appear synchronously with RP. Gastrointestinal involvement is a serious and prevalent complication of SSc, and the oesophagus is the most frequently affected organ. Both limited and diffuse cutaneous SSc involve internal organs, with the involvement of the gastrointestinal tract as a leading cause of morbidity. At present, treatment is mainly symptomatic with no disease-modifying drugs.
系统性硬化症（SSc）是一种病因不明的自身免疫性疾病，发病机制复杂，可累及多个器官。其特征是血管和免疫异常，导致皮肤和内脏器官纤维化。这是一种相当罕见的疾病，患病率约为每10万人中20例左右。该疾病会导致临床表现各异且病程不同。系统性硬化症通常始于雷诺现象（RP）的发作，随后出现皮肤硬化和内脏器官受累，尽管它可能与RP同时出现。胃肠道受累是系统性硬化症严重且常见的并发症，食管是最常受累的器官。局限性和弥漫性皮肤型系统性硬化症均会累及内脏器官，其中胃肠道受累是发病的主要原因。目前，治疗主要是对症治疗，尚无改善病情的药物。
