Morbidity and mortality in systemic lupus erythematosus during a 10-year period -: A comparison of early and late manifestations in a cohort of 1,000 patients

Morbidity and mortality in systemic lupus erythematosus during a 10-year period -: A comparison of early and late manifestations in a cohort of 1,000 patients
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DOI:
10.1097/01.md.0000091181.93122.55
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发表时间:
2003-09-01
期刊:
影响因子:
1.6
通讯作者:
Hughes, GRV
Hughes, GRV
中科院分区:
医学4区
文献类型:
--
作者:
Cervera, R;Khamashta, MA;Hughes, GRV

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在本研究中,我们评估了10年期间系统性红斑狼疮(SLE)发病和死亡的主要原因的频率和特征,并比较了早期表现与疾病发展后期出现的频率。1990年,我们开始了一项来自7个欧洲国家的1000名患者的多中心研究。所有人在进入研究时都有病史记录,并接受了医学访谈和常规全身检查,在随后的10年(1990-2000年)中,所有人都由同一位医生进行了前瞻性随访。共有481例(48.1%)患者在10年的任何时间出现1次或1次以上的关节炎发作,311例(31.1%)患者出现疟疾疹,279例(27.9%)出现活动性肾病,194例(19.4%)出现神经系统受累,166例(16.6%)出现发热,163例(16.3%)出现雷诺现象,160例(16.0%)出现浆膜炎(胸膜炎和/或心包炎),134例(13.4%)出现血小板减少,92例(9.2%)出现血栓形成。将随访前5年(1990-1995)的临床表现与随访后5年(1995-2000)的临床表现进行比较,发现大多数临床表现在随访前5年更为频繁。在1000例患者中,360例(36%)出现感染,169例(16.9%)出现高血压,121例(12.1%)出现骨质疏松,81例(8.1%)出现免疫抑制剂引起的细胞减少。23例(2.3%)患者发生恶性肿瘤;最常见的原发部位是子宫和乳房。68例(6.8%)患者死亡,最常见的死亡原因同样分为活动性SLE(26.5%)、血栓形成(26.5%)和感染(25%)。10年生存率为92%。研究开始时出现肾病的患者生存率较低(88%对94%,p = 0.045)。将最初5年随访期间(1990-1995)的死亡原因与随后5年(1995-2000)的死亡原因进行比较,活动性SLE和感染(各占28.9%)似乎是最初5年最常见的死亡原因,而血栓形成(26.1%)成为最后5年最常见的死亡原因。综上所述,大多数SLE炎症表现在疾病长期发展后似乎不太常见,这可能反映了治疗的效果以及许多患者疾病的逐渐缓解。同时,血栓形成事件的作用越来越明显,影响SLE的发病率和死亡率。
In the present study, we assessed the frequency and characteristics of the main causes of morbidity and mortality in systemic lupus erythematosus (SLE) during a 10-year period and compared the frequency of early manifestations with those that appeared later in the evolution of the disease. In 1990, we started a multicenter study of 1,000 patients from 7 European countries. All had medical histories documented and underwent medical interview and routine general physical examination when entered in the study, and all were followed prospectively by the same physicians during the ensuing 10 years (1990-2000).A total of 481 (48.1%) patients presented 1 or more episodes of arthritis at any time during the 10 years, 311 (31.1%) patients had malar rash, 279 (27.9%) active nephropathy, 194 (19.4%) neurologic involvement, 166 (16.6%) fever, 163 (16.3%) Raynaud phenomenon, 160 (16.0%) serositis (pleuritis and/or pericarditis), 134 (13.4%) thrombocytopenia, and 92 (9.2%) thrombosis. When the prevalences of the clinical manifestations during the initial 5 years of follow-up (1990-1995) were compared with those during the ensuing 5 years (1995-2000), most manifestations were found to be more frequent during the initial 5 years. Of the 1,000 patients, 360 (36%) presented infections, 169 (16.9%) hypertension, 121 (12.1%) osteoporosis, and 81 (8.1%) cytopenia due to immunosuppressive agents. Twenty-three (2.3%) patients developed malignancies; the most frequent primary localizations were the uterus and the breast. Sixty-eight (6.8%) patients died, and the most frequent causes of death were similarly divided between active SLE (26.5%), thromboses (26.5%), and infections (25%). A survival probability of 92% at 10 years was found. A lower survival probability was detected in those patients who presented at the beginning of the study with nephropathy (88% versus 94% in patients without nephropathy, p = 0.045). When the causes of death during the initial 5 years of follow-up (1990-1995) were compared with those during the ensuing 5 years (1995-2000), active SLE and infections (28.9% each) appeared to be the most common causes during the initial 5 years, while thromboses (26.1%) became the most common cause of death during the last 5 years.In conclusion, most of the SLE inflammatory manifestations appear to be less common after a long-term evolution of the disease, probably reflecting the effect of therapy as well as the progressive remission of the disease in many patients. Meanwhile, a more prominent role of thrombotic events is becoming evident, affecting both morbidity and mortality in SLE.