Jerjen, Rebekka; Nikpour, Mandana; Krieg, Thomas; Denton, Christopher P; Saracino, Amanda M; (2022) Systemic sclerosis in adults. Part I: Clinical features and pathogenesis. Journal of The American Academy of Dermatology , 87 (5) pp. 937-954. 10.1016/j.jaad.2021.10.065.

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Abstract

Systemic sclerosis (SSc; also referred to as systemic scleroderma or scleroderma), is a rare, complex immune-mediated connective tissue disease (CTD) characterized by progressive skin fibrosis and other clinically heterogenous features. The etiopathogenesis of SSc involves vasculopathy and immune system dysregulation occurring on a permissive genetic and epigenetic background, ultimately leading to fibrosis. Recent developments in our understanding of disease specific autoantibodies and bioinformatic analyses has led to reconsideration of the purely clinical classification of diffuse and limited cutaneous SSc subgroups. Autoantibody profiles are predictive of skin and internal organ involvement and disease course. Early diagnosis of SSc, with commencement of disease modifying treatment, has the potential to improve patient outcomes. Many early presenting clinical manifestations, signs of disease progression and activity in SSc are cutaneous, meaning dermatologists can and should play a key role in the diagnosis and management of this significant condition. The first article in this continuing medical education (CME) series discusses the epidemiology, clinical characteristics and pathogenesis of SSc in adults, with an emphasis on skin manifestations, the important role of dermatologists in recognising these, and their correlation with systemic features and disease course.

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