Bodolay E, Csiki Z, Szekanecz Z, Ben T, Kiss E, Zeher M, Szücs G, Dankó K, Szegedi G
Division of Immunology, 1st Department of Medicine, University of Debrecen, Medical and Health Science Center, Debrecen, Hungary.
Clin Exp Rheumatol. 2003 May-Jun;21(3):313-20.
To determine the clinical symptoms and the panel of autoantibodies of patients with early undifferentiated connective tissue disease (UCTD) followed for at least 1 year.
716 UCTD patients with manifestations suggestive but not diagnostic of specific connective tissue disease (CTD) were recruited and followed up between 1994-1999. The patients with early UCTD were subdivided into those with isolated Raynaud's phenomenon (RP) (50 patients), unexplained polyarthritis (31 patients) and "true" UCTD (665 patients). UCTD was diagnosed on the basis of clinical manifestations suggestive of a connective tissue disease and the presence of at least one non-organ specific autoantibody. The patients' sera were tested for anti-nuclear (ANA), as well as for nine different specific autoantibodies (anti-dsDNA, -Sm, -RNP, -SSA, -SSB, -Scl-70, -centromere, -Jo1 and -PM-Scl).
The most common clinical manifestations of UCTD included RP, arthritis/arthralgias, pleuritis/pericarditis, sicca symptoms, cutaneous involvement (photosensitivity, rash), central nervous symptoms, peripheral neuropathy, fever, vasculitis, less pulmonary involvement and myositis. 230 of the 665 true UCTD patients (34.5%) developed a defined CTD (28 systemic lupus erythematosus [SLE], 26 mixed connective tissue disease [MCTD], 19 progressive systemic sclerosis [PSS], 45 Sjögren's syndrome, 3 polymyositis/dermatomyositis [PM/DM], 87 rheumatoid arthritis [RA], and 22 systemic vasculitis. 435 of 665 patients (65.4%) remained in the UCTD state, and 82 of 665 patients (12.3%) achieved complete remission with symptoms not reappearing within the 5-year period. The highest probability of evolution to a defined CTD was during the first 2 years after onset: of 230 UCTD patients 183 (79.5%) developed major organ symptoms and signs. In particular skin and cardiac complications seemed to spread during the follow-up period in those patients who progressed to SLE. The condition of 18/50 patients with isolated RP evolved to UCTD and 3 of 31 patients with unexplained polyarthritis progressed to definite CTD (2 patients RA and one MCTD).
In our study most of the UCTD patients did not develop a definite CTD, but during the follow-up period we found new clinical and serological manifestations. One-third of the UCTD patients showed progress into different types of specific CTD.
确定随访至少1年的早期未分化结缔组织病(UCTD)患者的临床症状和自身抗体谱。
招募了716例有提示特定结缔组织病(CTD)但不能确诊的表现的UCTD患者,并在1994年至1999年期间进行随访。早期UCTD患者被细分为孤立性雷诺现象(RP)患者(50例)、不明原因多关节炎患者(31例)和“真正的”UCTD患者(665例)。UCTD的诊断基于提示结缔组织病的临床表现以及至少一种非器官特异性自身抗体的存在。检测患者血清中的抗核抗体(ANA)以及九种不同的特异性自身抗体(抗双链DNA、抗Sm、抗RNP、抗SSA、抗SSB、抗Scl-70、抗着丝点、抗Jo-1和抗PM-Scl)。
UCTD最常见的临床表现包括RP、关节炎/关节痛、胸膜炎/心包炎、干燥症状、皮肤受累(光敏性、皮疹)、中枢神经症状、周围神经病变、发热、血管炎、较少的肺部受累和肌炎。665例真正的UCTD患者中有230例(34.5%)发展为明确的CTD(28例系统性红斑狼疮[SLE]、26例混合性结缔组织病[MCTD]、19例进行性系统性硬化症[PSS]、45例干燥综合征、3例多发性肌炎/皮肌炎[PM/DM]、87例类风湿关节炎[RA]和22例系统性血管炎)。665例患者中有435例(65.4%)仍处于UCTD状态,665例患者中有82例(12.3%)实现完全缓解,5年内症状未再出现。发病后头2年发展为明确CTD的可能性最高:230例UCTD患者中有183例(79.5%)出现主要器官症状和体征。特别是皮肤和心脏并发症在进展为SLE的患者随访期间似乎有所增加。50例孤立性RP患者中有18例病情发展为UCTD,31例不明原因多关节炎患者中有3例进展为明确的CTD(2例RA和1例MCTD)。
在我们的研究中,大多数UCTD患者未发展为明确的CTD,但在随访期间我们发现了新的临床和血清学表现。三分之一的UCTD患者进展为不同类型的特定CTD。