Vaz C C, Couto M, Medeiros D, Miranda L, Costa J, Nero P, Barros R, Santos M J, Sousa E, Barcelos A, Inês L
Department of Rheumatology, Coimbra University Hospital, Praceta Prof. Mota Pinto, Coimbra 3000-075, Portugal.
Clin Rheumatol. 2009 Aug;28(8):915-21. doi: 10.1007/s10067-009-1175-2. Epub 2009 Apr 24.
The purpose of this study was to characterize the clinical and serological features of a large cohort of patients with antinuclear antibody (ANA) positive undifferentiated connective tissue disease (UCTD). Consecutive patients with UCTD, followed up at the Rheumatology Clinic of the participating centers, were included. Data from these patients were obtained by clinical evaluation and chart review. All patients were diagnosed as having UCTD on basis of the following criteria: positive ANA plus at least one clinical feature of connective tissue disease, but not fulfilling classification criteria for any differentiated connective tissue disease. One hundred eighty-four patients were studied (female patients-94.5%; mean age at time of evaluation-47 years). The most prevalent manifestations were arthralgia (66%), arthritis (32%), Raynaud's phenomenon (30%), sicca symptoms (30%), and leukopenia (19%). The prevalence of ANA was 100%, anti-SSA 20%, anti-dsDNA 14%, and anti-SSB 7%. Patients with anti-dsDNA/anti-Sm, anticentromere/anti-Scl70, or anti-SSA/anti-SSB antibodies more frequently presented a set of manifestations close to systemic lupus erythematosus (SLE), systemic sclerosis, or Sjögren syndrome, respectively. We analyze a large cohort of UCTD. Seventy-two percent of these UCTD patients present lupus-, scleroderma-, or Sjögren-like features but do not fulfill classification criteria and mostly present a mild disease.
本研究的目的是描述一大群抗核抗体(ANA)阳性的未分化结缔组织病(UCTD)患者的临床和血清学特征。纳入了在参与中心的风湿病诊所接受随访的连续UCTD患者。通过临床评估和病历审查获取这些患者的数据。所有患者均根据以下标准被诊断为UCTD:ANA阳性加至少一项结缔组织病的临床特征,但不符合任何分化型结缔组织病的分类标准。对184例患者进行了研究(女性患者占94.5%;评估时的平均年龄为47岁)。最常见的表现为关节痛(66%)、关节炎(32%)、雷诺现象(30%)、干燥症状(30%)和白细胞减少(19%)。ANA的阳性率为100%,抗SSA为20%,抗双链DNA为14%,抗SSB为7%。抗双链DNA/抗Sm、抗着丝点/抗Scl70或抗SSA/抗SSB抗体阳性的患者分别更常出现一组接近系统性红斑狼疮(SLE)、系统性硬化症或干燥综合征的表现。我们分析了一大群UCTD患者。这些UCTD患者中有72%表现出狼疮样、硬皮病样或干燥综合征样特征,但不符合分类标准,且大多病情较轻。