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十年系统性硬化症临床诊疗 - 深入了解与未来方向。

Ten years of a systemic sclerosis clinic in a tertiary referral centre - insights and future directions.

机构信息

Serviço de Reumatologia e Doenças Ósseas Metabólicas, Hospital de Santa Maria, Centro hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de Lisboa, Portugal

Unidade de Investigação em Reumatologia, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa, Portugal

出版信息

Acta Reumatol Port. 2021 Jul-Sep;46(3):257-265.

Abstract

Systemic sclerosis (SSc) is an uncommon condition, with a wide range of manifestations, characterized by specific antibody production, vasculopathy and fibrosis of the skin and other internal organs. It is a complex disease, which is estimated to be rare in Portugal, although specific incidence data are missing. The aetiology of SSc remains unknown, but is likely to be multifactorial, involving genetic and environmental aspects. Its management is challenging and often requires a multidisciplinary approach. In 2011, we established a dedicated outpatient clinic for patients with SSc. Clinical data of every patient with a confirmed diagnosis of SSc is prospectively registered in Reuma.pt/SSc. In this manuscript, we aim to describe the general functioning of our SSc outpatient clinic, and to characterise the population of patients with SSc who are followed herein.

摘要

系统性硬化症(SSc)是一种罕见的疾病,表现多样,其特征为特异性抗体产生、血管病变和皮肤及其他内脏器官纤维化。这是一种复杂的疾病,据估计在葡萄牙较为罕见,尽管缺乏具体的发病数据。SSc 的病因仍不清楚,但可能是多因素的,涉及遗传和环境方面。其治疗具有挑战性,通常需要多学科方法。2011 年,我们为 SSc 患者设立了专门的门诊。Reuma.pt/SSc 前瞻性登记了每一位确诊为 SSc 的患者的临床数据。在本文中,我们旨在描述 SSc 门诊的一般运作情况,并对在此处就诊的 SSc 患者人群进行特征描述。

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