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The complexity of managing systemic sclerosis: screening and diagnosis

Identifieur interne : 000D03 ( Istex/Checkpoint ); précédent : 000D02; suivant : 000D04

The complexity of managing systemic sclerosis: screening and diagnosis

Auteurs : M. Matucci-Cerinic [France, Italie] ; V. Steen [France] ; P. Nash [France] ; E. Hachulla [France]

Source :

RBID : ISTEX:F1243A2583736C36FEEB3B3F206119E7D8DAC925

Abstract

The difficulties inherent in diagnosing, screening and treating SSc are reflected by the complex pathology of the disease, which involves the development of severe organ-based complications that reduce both quality of life and overall survival. Early detection and prompt treatment of such complications depend upon a successful and timely screening strategy, which, in turn, requires cooperation between disciplines and good collaborative links at all stages of the disease. Establishment of a disease registry for SSc may also be of benefit, as such registries facilitate longitudinal observation of trends in disease presentation, management and outcome. They may also help to determine potential risk factors and identify those patient subgroups that face the highest risk of developing disease. In patients with known or suspected SSc, a panel of disease-specific markers—such as autoantibodies, cell activation markers and markers of organ involvement—may help to establish the diagnosis and assess prognosis; however, changes in serum levels of such markers throughout the course of SSc should be interpreted with caution, as they may not always correlate with disease activity. Nail-fold capillaroscopy is a promising tool for SSc assessment and may provide useful diagnostic and prognostic information, although further research is required to clarify its role in evaluating disease evolution.

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DOI: 10.1093/rheumatology/ken482


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ISTEX:F1243A2583736C36FEEB3B3F206119E7D8DAC925

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<div type="abstract">The difficulties inherent in diagnosing, screening and treating SSc are reflected by the complex pathology of the disease, which involves the development of severe organ-based complications that reduce both quality of life and overall survival. Early detection and prompt treatment of such complications depend upon a successful and timely screening strategy, which, in turn, requires cooperation between disciplines and good collaborative links at all stages of the disease. Establishment of a disease registry for SSc may also be of benefit, as such registries facilitate longitudinal observation of trends in disease presentation, management and outcome. They may also help to determine potential risk factors and identify those patient subgroups that face the highest risk of developing disease. In patients with known or suspected SSc, a panel of disease-specific markers—such as autoantibodies, cell activation markers and markers of organ involvement—may help to establish the diagnosis and assess prognosis; however, changes in serum levels of such markers throughout the course of SSc should be interpreted with caution, as they may not always correlate with disease activity. Nail-fold capillaroscopy is a promising tool for SSc assessment and may provide useful diagnostic and prognostic information, although further research is required to clarify its role in evaluating disease evolution.</div>
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