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The complexity of managing systemic sclerosis: screening and diagnosis

机译：处理系统性硬化的复杂性：筛查和诊断

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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.
机译：该病的复杂病理反映了诊断，筛查和治疗SSc固有的困难，这涉及发展严重的基于器官的并发症，从而降低生活质量和总体生存率。对此类并发症的早期发现和及时治疗取决于成功和及时的筛查策略，而这又需要各学科之间的合作以及疾病各个阶段的良好协作关系。建立SSc的疾病登记册也可能有益，因为此类登记册有助于纵向观察疾病呈报，管理和结果的趋势。他们还可以帮助确定潜在的危险因素，并确定面临疾病风险最高的那些患者亚组。在患有已知或疑似SSc的患者中，一组疾病特异性标志物（例如自身抗体，细胞激活标志物和器官受累标志物）可能有助于建立诊断和评估预后。然而，在整个SSc过程中，此类标志物血清水平的变化应谨慎解释，因为它们可能并不总是与疾病活动相关。尽管需要进一步的研究来阐明其在评估疾病进展中的作用，但是钉状毛细管内窥镜检查是评估SSc的有前途的工具，并且可以提供有用的诊断和预后信息。

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  • 来源
    《Rheumatology》 |2009年第3期|p.8-13|共6页
  • 作者

    M. Matucci-Cerinic1 V. Steen2 P. Nash3 and E. Hachulla4;

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    1Department of Biomedicine, Centre DenoThe, Division of Rheumatology, AOUC University of Florence, Florence, Italy, 2Department of Medicine, Georgetown University, Washington DC, USA, 3Rheumatology Research Unit, Sunshine Coast Queensland Department of Medicine, University of Queensland, Queensland, Australia and 4Hôpital Claude-Huriez, University of Lille, Lille, France.;

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