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[未分化结缔组织病:578例患者随访5年的临床和血清学特征:疾病进程、预后及治疗]

[Undifferentiated connective tissue disease: clinical and serological profile of 578 patients followed for five years: disease course, prognosis and therapy].

作者信息

Bodolay Edit, Szegedi Gyula

机构信息

Debreceni Egyetem, Orvos- és Egészségtudományi Centrum, Országos Immunológiai Koordinációs Intézet.

出版信息

Orv Hetil. 2002 Feb 3;143(5):229-33.

Abstract

INTRODUCTION

Evolution of immunopathological diseases is usually slow and progressive. The term the undifferentiated connective tissue disease (UCTD) is used to describe the phase preceding a defined connective tissue diseases (CTD).

AIMS

The objective of this work was evaluate the clinical and serological profile of patients with UCTD, who had been followed between 1994-1999. They have investigated the frequency and the type the developed autoimmune diseases from UCTD.

PATIENTS

A total of 578 UCTD patients were evaluated.

RESULTS

In 143/578 patients (24.7%) with the UCTD differentiated to systemic connective tissue diseases (28 systemic lupus erythematosus, 26 mixed connective tissue disease, 19 progressive systemic sclerosis, 3 polymyositis/dermatomyositis, 45 Sjögren syndrome, and 22 systemic vasculitis). 86.7 percent (124/143) of the systemic connective disease developed in first two years of UCTD. The condition of 435/578 (75.2%) remained UCTD after 5 years, among them in 82 patients with UCTD was regression of the symptoms. The presence of the fever and anti-DNS antibodies correlated with SLE (P = 0.0104, Fisher exact test), arthritis/arthralgia and anti-RNP antibodies with MCTD (P = 0.0302), Raynaud phenomenon and ANA positivity with PSS (P = 0.0144), xerostomia/xerophtalmia and anti-SSA/SSB antibodies with Sjögren syndromes (P = 0.0144).

CONCLUSIONS

The UCTD in our patients seem to represents an dynamic phase, one part of the patients show progression to definite connective tissue diseases, one part show regression, and on part of the patients stay in UCTD phase.

摘要

引言

免疫病理疾病的演变通常较为缓慢且呈进行性发展。未分化结缔组织病(UCTD)这一术语用于描述明确的结缔组织病(CTD)之前的阶段。

目的

本研究的目的是评估1994年至1999年期间随访的UCTD患者的临床和血清学特征。他们调查了UCTD发展为自身免疫性疾病的频率和类型。

患者

共评估了578例UCTD患者。

结果

在578例UCTD患者中,有143例(24.7%)发展为系统性结缔组织病(28例系统性红斑狼疮、26例混合性结缔组织病、19例进行性系统性硬化症、3例多发性肌炎/皮肌炎、45例干燥综合征和22例系统性血管炎)。86.7%(124/143)的系统性结缔组织病在UCTD的头两年内发展。5年后,578例患者中有435例(75.2%)仍为UCTD,其中82例UCTD患者症状有所缓解。发热和抗双链DNA抗体的存在与系统性红斑狼疮相关(P = 0.0104,Fisher精确检验),关节炎/关节痛和抗核糖核蛋白抗体与混合性结缔组织病相关(P = 0.0302),雷诺现象和抗核抗体阳性与进行性系统性硬化症相关(P = 0.0144),口干/眼干和抗SSA/SSB抗体与干燥综合征相关(P = 0.0144)。

结论

我们患者中的UCTD似乎代表一个动态阶段,一部分患者发展为明确的结缔组织病,一部分患者症状缓解,还有一部分患者停留在UCTD阶段。

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