Abstract
Purpose of reviewAt the time of diagnosis, systemic sclerosis (SSc) is often well established with significant irreversible tissue and organ damage. Definitions of ‘early SSc’ have been proposed, which include the presence of SSc-associated autoantibodies. In addition, functional autoantibodies that are believed to be involved in SSc pathogenesis need to be considered. In this review, recent advances in the diagnostic utility and pathogenic role of autoantibodies in early SSc are summarized. Moreover, we propose a clinical care pathway illustrating how autoantibody testing along with key clinical features can be used to make an earlier diagnosis of SSc.Recent findingsRecent evidence has helped to develop a clearer understanding of the natural history, early clinical features, and autoantibodies that are predictors of SSc. The role of functional autoantibodies is leading to innovative approaches to evidence-based interventions and therapies that are based on mechanisms of disease.SummaryDespite substantial advances, the high morbidity and mortality that currently characterizes SSc can largely be attributed to a delay in diagnosis, gaps in our understanding of the role of autoantibodies in early disease, and limited effective therapeutic options. An early and accurate diagnosis of SSc and use of autoantibody testing embedded in evidence-based clinical care pathways will help improve SSc-associated clinical outcomes and healthcare expenditures.
Highlights
Systemic sclerosis (SSc) is a chronic, multisystem disorder that evolves through stages of early initiation, disease amplification, and later progression, all characterized by an overlapping triad of autoimmunity, microvascular abnormalities, and variable degrees of fibrosis [1&&]
Autoantibodies directed to nuclear components [antinuclear antibodies (ANAs)] were the first to be described only to be followed by an appreciation that cytoplasmic, cell membrane, and even extracellular components were included in the SSc B-cell repertoire [2,3&,4]
We propose a clinical care pathway highlighting the use SSc autoantibodies and key clinical features to help with the diagnosis and management of early disease
Summary
Systemic sclerosis (SSc) is a chronic, multisystem disorder that evolves through stages of early initiation (triggering events), disease amplification, and later progression, all characterized by an overlapping triad of autoimmunity, microvascular abnormalities, and variable degrees of fibrosis [1&&]. Autoantibodies directed to nuclear components [antinuclear antibodies (ANAs)] were the first to be described only to be followed by an appreciation that cytoplasmic, cell membrane, and even extracellular components were included in the SSc B-cell repertoire [2,3&,4]. In addition to their role as diagnostic biomarkers, there is increasing evidence that autoimmunity occurs early in disease, plays an important role in pathogenesis, and is correlated with end-organ manifestations [4,5&]. The work cannot be changed in any way or used commercially
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