Skin sclerosis is only of limited value to identify SSc patients with severe manifestations-an analysis of a distinct patient subgroup of the German Systemic Sclerosis Network (DNSS) Register
Skin sclerosis is only of limited value to identify SSc patients with severe manifestations-an analysis of a distinct patient subgroup of the German Systemic Sclerosis Network (DNSS) Register
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DOI：
10.1093/rheumatology/ken408
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发表时间：
2009-01-01
期刊：
影响因子：
5.5
通讯作者：
Riemekasten, G.
中科院分区：
文献类型：
--
作者：
Hanitsch, L. G.;Burmester, G. -R.;Riemekasten, G.
目标。在 SSc 中，诊断和分类主要基于皮肤硬化。在此，我们在一个大型多中心队列中调查了皮肤硬化在多大程度上反映了器官受累和其他临床症状。方法。对德国系统性硬化症网络 (DNSS) 登记册中总共 1200 名 SSc 患者（分类为 lcSSc 或 dcSSc）进行了分析，分析了他们的血清学特征、临床症状和与皮肤受累相关的器官表现（通过改良的 Rodnan 皮肤评分 (mRSS) 测量）。结果。具有不同 mRSS 的 SSc 患者的病程和大多数临床症状没有显着差异。他们表现出大多数器官表现的相似分布，例如肺动脉高压以及心脏、肾脏和神经系统受累。更严重的皮肤增厚被发现与肺纤维化和胃肠道症状以及指溃疡和肌肉骨骼受累有关。结论。在 SSc 患者中，潜在危及生命的并发症和对生活质量影响很大的临床症状的发生与皮肤硬化的程度无关。 mRSS 较低的 SSc 患者可能会被漏诊，或者治疗不充分。
Objectives. In SSc, diagnosis and classification is based mainly on skin sclerosis. Herein, we investigated in a large multicentre cohort, to what extent skin sclerosis reflects organ involvement and additional clinical symptoms.Methods. A total of 1200 SSc patients from the register of the German Systemic Sclerosis Network (DNSS), classified as either lcSSc or dcSSc, were analysed for their serological characteristics, clinical symptoms and organ manifestations in relation to skin involvement measured by the modified Rodnan skin score (mRSS).Results. SSc patients with different mRSS did not differ significantly in their disease duration and in most of the clinical symptoms. They showed a similar distribution of most organ manifestations such as pulmonary arterial hypertension as well as cardiac, renal and nervous system involvement. More severe skin thickening was found to be associated with pulmonary fibrosis and gastrointestinal symptoms, as well as with digital ulcers and musculoskeletal involvement.Conclusions. In patients with SSc, potentially life-threatening complications and clinical symptoms with high impact on the quality of life occur independently from the extent of skin sclerosis. The diagnosis in SSc patients with a low mRSS could be missed or they could be insufficiently treated.