Ziswiler Hans-Rudolf, Urech Romana, Balmer Judith, Ostensen Monika, Mierau Rudolf, Villiger Peter M
Department of Rheumatology, Clinical Immunology and Allergology, University Hospital, Bern, Switzerland.
Swiss Med Wkly. 2007 Oct 20;137(41-42):586-90. doi: 10.4414/smw.2007.11900.
To compare clinical diagnosis with two validated classification criteria for systemic sclerosis (SSc) in a cohort of Swiss patients with SSc and associated disorders.
Charts of 54 patients with SSc and associated disorders were reviewed and compared with data obtained at a thorough clinical examination using a standardised protocol (Raynaud's phenomen [RP], skin involvement, nailfold capillary microscopy and determination of autoantibody pattern).
According to patient records 6 patients had diffuse cutaneous SSc (dcSSc), 23 limited cutaneous SSc (lcSSc) and 20 were not classified. Two patients had mixed connective tissue disease (MCTD) and 3 overlap syndromes. At the time of clinical examination, 7 patients showed dcSSc (6 plus 1 patient originally classified as lcSSc), 26 lcSSc (20 plus 6 originally not classified) and 16 patients had severe RP which was arbitrarily classified as Raynaud's syndrome (RS). 15 of the latter 16 were antinuclear antibody positive and 7 exhibited pathological nailfold capillaries. On the basis of LeRoy and Medsger's criteria, 6 of these patients could be further classified as limited SSc (lSSc). Of 49 sera tested, 14 contained centromere antibodies at clinical examination, 16 Scl-70, 5 RNA-pol, 1 Ku, 12 antibodies with unknown specificity, and one serum was autoantibody negative.
A substantial number of patients with minor cutaneous manifestations do not fulfil ACR classification criteria, though they have typical clinical signs of SSc. Characteristic features in these patients are presence of Raynaud's phenomenon, antinuclear antibodies and pathological changes in nailfold capillary microscopy. Application of the diagnostic criteria recently proposed by LeRoy and Medsger makes it possible to name many of these patients. The use of these criteria is recommended for clinical management.
在一组瑞士系统性硬化症（SSc）患者及相关疾病患者中，比较临床诊断与两种已验证的SSc分类标准。
回顾了54例SSc及相关疾病患者的病历，并与使用标准化方案（雷诺现象[RP]、皮肤受累情况、甲襞毛细血管显微镜检查及自身抗体谱测定）进行全面临床检查时获得的数据进行比较。
根据患者记录，6例为弥漫性皮肤型SSc（dcSSc），23例为局限性皮肤型SSc（lcSSc），20例未分类。2例患有混合性结缔组织病（MCTD），3例为重叠综合征。在临床检查时，7例表现为dcSSc（6例加上1例原分类为lcSSc的患者），26例为lcSSc（20例加上6例原未分类的患者），16例有严重的RP，被随意分类为雷诺综合征（RS）。后16例中的15例抗核抗体阳性，7例甲襞毛细血管有病理改变。根据勒罗伊（LeRoy）和梅兹格（Medsger）的标准，这些患者中有6例可进一步分类为局限性SSc（lSSc）。在检测的49份血清中，临床检查时有14份含有着丝点抗体，16份含有Scl - 测定，5份含有RNA - pol，1份含有Ku，12份抗体特异性未知，1份血清自身抗体阴性。
相当一部分皮肤表现轻微的患者虽有SSc的典型临床体征，但不符合美国风湿病学会（ACR）分类标准。这些患者的特征性表现为存在雷诺现象、抗核抗体及甲襞毛细血管显微镜检查的病理改变。应用勒罗伊和梅兹格最近提出的诊断标准可使许多此类患者得以明确诊断。建议在临床管理中使用这些标准。
