Calderon J, Rodriguez-Valverde V, Sanchez Andrade S, Riestra J L, Gomez-Reyno J
Clin Rheumatol. 1984 Dec;3(4):483-92. doi: 10.1007/BF02031271.
Currently there are no widely accepted criteria for the diagnosis of MCTD. In this work we attempted to define the clinical profile of a group of 68 patients with anti nRNP antibodies, detected by immunoprecipitation in 0.6% agarose. The diagnosis of each collagen vascular disease was established in every patient, who met with the strict diagnostic criteria either at clinical presentation or during the follow-up period. Twenty-eight patients had SLE, 9 had classical erosive RA, three had PSS and one had PM. The only distinctive features in the group of SLE with anti nRNP was an increased incidence of anti Sm antibodies (p less than 0.05). In the RA group there was a trend towards a high frequency of Raynaud's phenomenon and swollen hands. At clinical presentation twenty-seven patients did not fulfil enough criteria to be diagnosed of any of the well-defined collagen vascular disease. They presented an undifferentiated syndrome, characterized clinically by Raynaud's phenomenon (100%), swollen hands (88.9%) and joint symptoms (88.9%), with scarce tendency of developing severe systemic manifestations. The main laboratory abnormalities in this group were hypergammaglobulinemia, mildly increased ESR, abnormal levels of CIC, negative anti nDNA and anti Sm antibodies, and the virtual absence of hypocomplementemia. During a clinical course of 96 +/- 72.5 months only one patient evolved into another collagen disease (SLE). The clinical course in the remaining cases, was stable improving with low doses of prednisone and/or NSAID. We suggest considering this undifferentiated syndrome as a distinct entity, for which the already classical term of MCTD could be reserved.
目前,尚无被广泛接受的混合性结缔组织病(MCTD)诊断标准。在本研究中,我们试图明确一组68例抗nRNP抗体患者的临床特征,这些抗体通过在0.6%琼脂糖中进行免疫沉淀检测得出。对每位符合严格诊断标准的患者,无论是在临床表现时还是随访期间,均确立了每种胶原血管病的诊断。28例患者患有系统性红斑狼疮(SLE),9例患有典型侵蚀性类风湿关节炎(RA),3例患有进行性系统性硬化症(PSS),1例患有多发性肌炎(PM)。抗nRNP的SLE组中唯一的独特特征是抗Sm抗体发生率增加(p小于0.05)。在RA组中,雷诺现象和手部肿胀的发生率有升高趋势。在临床表现时,27例患者不符合任何明确的胶原血管病诊断标准。他们表现为未分化综合征,临床特征为雷诺现象(100%)、手部肿胀(88.9%)和关节症状(88.9%),发展为严重全身表现的倾向较少。该组主要的实验室异常包括高球蛋白血症、血沉轻度升高、循环免疫复合物(CIC)水平异常、抗nDNA和抗Sm抗体阴性,以及几乎没有补体低下。在96±72.5个月的临床病程中,仅有1例患者发展为另一种胶原病(SLE)。其余病例的临床病程稳定,使用低剂量泼尼松和/或非甾体抗炎药(NSAID)后病情改善。我们建议将这种未分化综合征视为一种独特的疾病实体,可保留已有的MCTD这一经典术语来称呼它。