Williams H J, Alarcon G S, Joks R, Steen V D, Bulpitt K, Clegg D O, Ziminski C M, Luggen M E, St Clair E W, Willkens R F, Yarboro C, Morgan J G, Egger M J, Ward J R
Cooperative Systemic Studies of the Rheumatic Diseases, University of Utah School of Medicine, Salt Lake City, USA.
J Rheumatol. 1999 Apr;26(4):816-25.
(1) To review the diagnoses after 10 years in patients who were identified within 12 months of the onset of well established and undifferentiated connective tissue diseases (CTD). (2) To examine the death rates and disease remissions in these patients.
This inception cohort of 410 patients had less than one year of signs and/or symptoms of CTD. Diagnoses of rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and poly/dermatomyositis (PM/DM) were made in 197 patients using accepted diagnostic and classification criteria. Diagnoses of undifferentiated CTD were made in 213 patients. These latter patients were placed in 3 categories: isolated Raynaud's phenomenon (RP), unexplained polyarthritis (UPA), and undifferentiated CTD (UCTD), defined as meeting at least 3 of 11 specific manifestations of CTD. The diagnoses and remissions in all patients after 10 years were determined.
Patients with well established CTD tended to remain with the original diagnosis. The 10 year survival was at least 87% in all diagnostic categories, with the exception of SSc, in which it was 56%. The progression of UPA to RA occurred infrequently. The presence of antinuclear antibodies suggested that UPA may develop additional symptoms and/or a specific diagnosis, and RP in these patients increased the likelihood of progressing to UCTD or a specific well established CTD. Ten percent of patients with RP progressed to SSc. In patients with UCTD, joint pain/tenderness and swelling counts were associated with progression to other diagnoses including RA, while either serositis, malar rash, or discoid lupus suggested the eventual diagnosis of SLE.
The survival of patients with SSc was poor, with most dying early in the course of their disease. Remissions were seen in all groups of patients except SSc. The remissions were sometimes transient in SLE. Undifferentiated disease at initial examination within 12 months of onset usually remains undifferentiated.
（1）回顾确诊为明确的和未分化的结缔组织病（CTD）发病12个月内确诊的患者10年后的诊断情况。（2）研究这些患者的死亡率和疾病缓解情况。
这个起始队列包含410例CTD体征和/或症状出现时间少于1年的患者。根据公认的诊断和分类标准，197例患者被诊断为类风湿关节炎（RA）、系统性红斑狼疮（SLE）、系统性硬化症（SSc）和多/皮肌炎（PM/DM）。213例患者被诊断为未分化CTD。后一组患者分为3类：孤立性雷诺现象（RP）、不明原因多关节炎（UPA）以及未分化CTD（UCTD），UCTD定义为符合CTD 11种特定表现中的至少3种。确定了所有患者10年后的诊断和缓解情况。
确诊为明确CTD的患者倾向于维持原诊断。除SSc组10年生存率为56%外，所有诊断类别10年生存率至少为87%。UPA进展为RA的情况很少见。抗核抗体的存在提示UPA可能出现更多症状和/或特定诊断，这些患者中的RP增加了进展为UCTD或特定明确CTD的可能性。10%的RP患者进展为SSc。在UCTD患者中，关节疼痛/压痛和肿胀计数与进展为包括RA在内的其他诊断相关，而浆膜炎、颊部皮疹或盘状狼疮提示最终诊断为SLE。
SSc患者生存率低，大多数在疾病早期死亡。除SSc组外，所有患者组均出现缓解。SLE组的缓解有时是短暂的。发病12个月内初次检查时的未分化疾病通常仍保持未分化状态。
