Ciancarella Claudia, Ceccarelli Fulvia, Picciariello Licia, Natalucci Francesco, Celia Alessandra Ida, Garufi Cristina, Mancuso Silvia, Tripodi Giuseppe, Truglia Simona, Gattamelata Angelica, Spinelli Francesca Romana, Alessandri Cristiano, Conti Fabrizio
Lupus Clinic, Rheumatology, Department of Clinical, Internal, Anesthesiologic and Cardiovascular, Sciences, Sapienza University of Rome, Viale del Policlinico 155, 00161, Rome, Italy.
Clin Exp Med. 2025 May 1;25(1):134. doi: 10.1007/s10238-025-01668-1.
Undifferentiated connective tissue disease (UCTD) is a condition characterized by serological evidence of autoimmunity and occurrence of clinical symptoms suggestive for systemic autoimmune diseases, yet not fulfilling specific classification/diagnostic criteria. In the present longitudinal, observational, retrospective study, we aimed at analysing the evolution of UCTD course, focussing on the impact of 2019 EULAR/ACR classification criteria for systemic lupus erythematosus (SLE). Since 2008 we consecutively collected data about UCTD patients. All subjects were evaluated every six months, to record the development of clinical and laboratory features suggestive for specific autoimmune diseases. Finally, we retrospectively applied the 2019 EULAR/ACR SLE classification criteria at the first and last visit in our outpatient clinic. All the patients included in the study had been evaluated at our Lupus Clinic before the release of 2019 EULAR/ACR criteria. We included 201 UCTD patients [F/M 191/10, median age at first visit 46 years (IQR 21), median disease duration at first visit 3 years (IQR 9)]. At the first visit, 27 patients (13.4%) already met 2019 EULAR/ACR SLE classification criteria. Logistic regression analysis demonstrated the association between SLE classification and thrombocytopenia, anti-dsDNA/anti-Sm positivity, low C4 levels, joint involvement. During a mean observation period of 45.9 ± 35.6 months, 18.9% of patients were lost to follow-up, while 141 patients were followed. At last visit, additional 11 patients (7.8%) could be classified as having SLE. A relevant proportion of UCTD patients could be reclassified as having SLE according to the most recent classification criteria. Thrombocytopenia, anti-DNA/anti-Sm positivity and low C4 levels represent the most associated factors.
未分化结缔组织病（UCTD）是一种以自身免疫的血清学证据和提示系统性自身免疫性疾病的临床症状发作为特征，但不符合特定分类/诊断标准的疾病。在本纵向、观察性、回顾性研究中，我们旨在分析UCTD病程的演变，重点关注2019年欧洲抗风湿病联盟（EULAR）/美国风湿病学会（ACR）系统性红斑狼疮（SLE）分类标准的影响。自2008年以来，我们连续收集了UCTD患者的数据。所有受试者每六个月接受一次评估，以记录提示特定自身免疫性疾病的临床和实验室特征的发展情况。最后，我们在门诊首次就诊和末次就诊时回顾性应用2019年EULAR/ACR SLE分类标准。纳入研究的所有患者在2019年EULAR/ACR标准发布之前已在我们的狼疮诊所接受评估。我们纳入了201例UCTD患者[女性/男性191/10，首次就诊时的中位年龄46岁（四分位间距21），首次就诊时的中位病程3年（四分位间距9）]。在首次就诊时，27例患者（13.4%）已符合2019年EULAR/ACR SLE分类标准。逻辑回归分析表明SLE分类与血小板减少、抗双链DNA/抗Sm阳性、低C4水平、关节受累之间存在关联。在平均45.9±35.6个月的观察期内，18.9%的患者失访，而141例患者得到随访。在末次就诊时，另有11例患者（7.8%）可被分类为患有SLE。根据最新分类标准，相当一部分UCTD患者可被重新分类为患有SLE。血小板减少、抗DNA/抗Sm阳性和低C4水平是最相关的因素。
