Rheumatology Unit, Department of Internal Medicine, University of Pisa, Italy.
Autoimmun Rev. 2011 Mar;10(5):256-8. doi: 10.1016/j.autrev.2010.09.013. Epub 2010 Sep 21.
Conditions characterized by the presence of clinical and serological manifestations suggestive of systemic autoimmune diseases but not fulfilling the classification criteria for defined connective tissue disease (CTD) are common in clinical practice and are indicated as undifferentiated (U) CTDs. Although epidemiological data are not available in the literature, up to 50% of the patients with an undifferentiated CTD of less than one year of duration were reported. The majority of patients suffering from UCTDs are young females, do not evolve into full blown CTD and are stable over time displaying mild clinical manifestations and single autoantibody profile. The early identification of stable UCTD is important for therapeutic and prognostic reasons and for investigating the pathogenic significance of autoantibodies or the role of the genetic background. Classification criteria for stable UCTDs are still matter of debate.
临床上存在一些以临床和血清学表现为特征、提示系统性自身免疫性疾病的情况,但不符合明确结缔组织病(CTD)的分类标准,这些情况较为常见。尽管文献中尚无流行病学数据,但有报道称,1 年内出现未分化 CTD 的患者中,多达 50%属于这种情况。大多数患有未分化 CTD 的患者为年轻女性,不会发展为典型的 CTD,而且随着时间的推移会保持稳定,表现为轻度临床症状和单一自身抗体谱。出于治疗和预后原因以及为了研究自身抗体的发病意义或遗传背景的作用,早期识别稳定的未分化 CTD 非常重要。稳定未分化 CTD 的分类标准仍存在争议。