Mosca M, Tani C, Neri C, Baldini C, Bombardieri S
Rheumatology Unit, Department of Internal Medicine, University of Pisa, Via Roma, 67, 56126 Pisa, Italy.
Autoimmun Rev. 2006 Nov;6(1):1-4. doi: 10.1016/j.autrev.2006.03.004. Epub 2006 Apr 19.
The term undifferentiated connective tissue diseases is used to define conditions characterized by the presence of signs and symptoms suggestive of a systemic autoimmune disease that do not satisfy the classificative criteria for defined connective tissue diseases (CTD) such as systemic lupus erythematosus (SLE), Sjögren's syndrome (SS), rheumatoid arthritis (RA) and others. A small percentage of patients presenting with an undifferentiated profile will develop during the first year follow up of a full blown CTD, however an average of 75% will maintain an undifferentiated clinical course. These patients may be defined as having a stable undifferentiated connective tissue diseases (UCTD). The most characteristic symptoms of UCTD are represented by arthritis and arthralgias, Raynaud's phenomenon, leukopenia, while neurological and kidney involvement are virtually absent. Eighty percent of these patients have a single autoantibody specificity, more frequently anti-Ro and anti-RNP antibodies. Stable UCTD are considered as distinct clinical entities and therefore it has been proposed to define those conditions as UCTD. Classificative criteria have also been proposed and a work to better define them is still under way.
未分化结缔组织病这一术语用于定义那些具有提示系统性自身免疫性疾病的体征和症状，但不满足已定义的结缔组织病（CTD）如系统性红斑狼疮（SLE）、干燥综合征（SS）、类风湿关节炎（RA）等分类标准的疾病状态。一小部分表现为未分化特征的患者在第一年随访期间会发展为典型的CTD，然而平均75%的患者将维持未分化的临床病程。这些患者可被定义为患有稳定的未分化结缔组织病（UCTD）。UCTD最典型的症状表现为关节炎和关节痛、雷诺现象、白细胞减少，而几乎不存在神经和肾脏受累情况。这些患者中有80%具有单一自身抗体特异性，最常见的是抗Ro和抗RNP抗体。稳定的UCTD被视为独特的临床实体，因此有人提议将这些疾病状态定义为UCTD。也有人提出了分类标准，并且一项旨在更好地定义它们的工作仍在进行中。
