Tselios K, Gladman D D, Touma Z, Su J, Anderson N, Urowitz M B
Centre for Prognosis Studies in Rheumatic Diseases, Toronto Lupus Clinic, Toronto, Canada.
Lupus. 2019 Jan;28(1):114-122. doi: 10.1177/0961203318817132. Epub 2018 Dec 8.
Disease activity in systemic lupus erythematosus follows three different courses: long quiescent, relapsing remitting and persistently active. However, the patterns of disease course since diagnosis are not known. This study aimed to assess the prevalence and characteristics of such patterns over 10 years.
The inception cohort of the Toronto Lupus Clinic (≥10 year follow up, between visit interval ≤18 months) was investigated. Prolonged remission was defined as a clinical Systemic Lupus Erythematosus Disease Activity Index 2000 = 0 achieved within 5 years of enrolment and maintained for ≥10 years. The relapsing-remitting pattern was defined based on ≥2 remission periods (clinical Systemic Lupus Erythematosus Disease Activity Index 2000 = 0 for two consecutive visits). Patients with no remission were categorized as persistently active. Groups were compared for baseline characteristics, cumulative damage, flare rate, mortality and certain co-morbidities.
Of 267 patients, 27 (10.1%) achieved prolonged remission, 180 (67.4%) relapsing-remitting and 25 (9.4%) persistently active. In total, 35 (13.1%) had only one remission period (hybrid). At enrollment, there were no differences regarding clinical and immunological variables. At 10 years, persistently active patients had accumulated significantly more damage than the prolonged remission and relapsing-remitting patients. Being of Black race and higher adjusted mean Systemic Lupus Erythematosus Disease Activity Index 2000 over the first 2 years were associated with a more severe disease course. Relapsing-remitting and persistently active patients had an increased flare rate and accrued more osteoporosis, osteonecrosis and cardiovascular events.
Approximately 70% of systemic lupus erythematosus patients followed a relapsing-remitting course, whereas 10% displayed prolonged remission and another 10% a persistently active course. Early response to treatment was associated with a less severe course and better prognosis.
系统性红斑狼疮的疾病活动呈现三种不同病程：长期缓解、复发缓解和持续活动。然而，自确诊以来的疾病病程模式尚不清楚。本研究旨在评估10年间这些病程模式的患病率及特征。
对多伦多狼疮诊所的起始队列（随访≥10年，就诊间隔≤18个月）进行调查。长期缓解定义为入组后5年内达到临床系统性红斑狼疮疾病活动指数2000=0并维持≥10年。复发缓解模式基于≥2次缓解期（连续两次就诊临床系统性红斑狼疮疾病活动指数2000=0）来定义。无缓解的患者归类为持续活动。比较各组的基线特征、累积损伤、疾病发作率、死亡率及某些合并症。
267例患者中，27例（10.1%）达到长期缓解，180例（67.4%）为复发缓解，25例（9.4%）为持续活动。共有35例（13.1%）仅有1次缓解期（混合型）。入组时，临床和免疫学变量无差异。10年时，持续活动的患者累积的损伤明显多于长期缓解和复发缓解的患者。黑人种族以及最初2年调整后的平均系统性红斑狼疮疾病活动指数2000较高与更严重的疾病病程相关。复发缓解和持续活动的患者疾病发作率增加，骨质疏松、骨坏死和心血管事件累积更多。
约70%的系统性红斑狼疮患者病程为复发缓解型，而10%表现为长期缓解，另有10%为持续活动型。对治疗的早期反应与较不严重的病程及更好的预后相关。
