Hao Yanjie, Hansen Dylan, Louthrenoo Worawit, Chen Yi-Hsing, Cho Jiacai, Lateef Aisha, Hamijoyo Laniyati, Luo Shue Fen, Wu Yeong-Jian, Navarra Sandra, Zamora Leonid, Li Zhanguo, Sockalingam Sargunan, Katsumata Yasuhiro, Harigai Masayoshi, Ji Lanlan, Zhang Zhuoli, Chan Madelynn, Kikuchi Jun, Takeuchi Tsutomu, Bae Sang-Cheol, Goldblatt Fiona, O'Neill Sean, Ng Kristine Pek Ling, Basnayake B M D B, Tugnet Nicola, Ohkubo Naoaki, Tanaka Yoshiya, Tee Cherica, Tee Michael, Lau C S, Li Ning, Golder Vera, Hoi Alberta, Kandane-Rathnayake Rangi, Morand Eric, Oon Shereen, Nikpour Mandana
The University of Melbourne and St Vincent's Hospital Melbourne, Melbourne, Victoria, Australia, and Peking University First Hospital, Beijing, China.
St Vincent's Hospital Melbourne, Melbourne, Victoria, Australia.
ACR Open Rheumatol. 2025 Mar;7(3):e70007. doi: 10.1002/acr2.70007.
In contrast to relapsing-remitting patterns, persistently active disease (PAD) is a disease activity pattern in patients with systemic lupus erythematosus (SLE) that is inadequately studied. We sought to identify the frequency and determinants of flare and PAD in SLE.
Flare was defined using the Safety of Estrogens in Lupus Erythematosus National Assessment version of the Systemic Lupus Erythematosus Disease Activity Index (SELENA-SLEDAI flare index), and PAD was defined as an SLEDAI-2K score of ≥4, excluding serology only, on two or more consecutive visits with a maximum six-month interval. Multivariable logistic regression was used to develop predictive models for flare and PAD, which were tested in an independent validation subset.
Among 3,811 patients over 2.8 (interquartile range 1.0-5.3) years of follow-up, 2,142 (56.2%) experienced flare and 1,786 (46.9%) had PAD, with 368 (9.7%) experiencing PAD but not flare. The most common flare features were nephritis and arthritis, whereas PAD was most commonly characterized by renal or mucocutaneous activity. After adjusting for prednisone dose and use of antimalarials and immunosuppressants, low gross domestic product in country of residence, smoking, arthritis, nephritis, and low complement levels were predictive for flare, whereas being in a low disease activity state for ≥50% of follow-up time (LLDAS50) was a protective factor. Renal activity and higher time-adjusted mean SLEDAI-2K were predictive of PAD, whereas LLDAS50 was protective. The models developed gave 72.1% and 83.8% correct classification of flare and PAD, respectively, in the validation cohort.
Both flare and PAD are common disease activity patterns in SLE; both predict organ damage accrual but differ in disease features and predictive factors. Because 9.7% of patients experience PAD but not flare, flare measures alone do not adequately capture all patients in whom disease control is suboptimal.
与复发缓解型模式不同，持续活动性疾病（PAD）是系统性红斑狼疮（SLE）患者中一种研究不足的疾病活动模式。我们试图确定SLE中疾病复发和PAD的频率及决定因素。
使用《红斑狼疮雌激素安全性全国评估》版系统性红斑狼疮疾病活动指数（SELENA-SLEDAI复发指数）定义疾病复发，PAD定义为在间隔最长6个月的两次或更多次连续就诊时，SLEDAI-2K评分≥4（仅排除血清学指标）。采用多变量逻辑回归建立疾病复发和PAD的预测模型，并在独立验证子集中进行检验。
在3811例患者超过2.8年（四分位间距1.0 - 5.3年）的随访中，2142例（56.2%）出现疾病复发，1786例（46.9%）有PAD，其中368例（9.7%）有PAD但未出现疾病复发。最常见的疾病复发特征是肾炎和关节炎，而PAD最常见的特征是肾脏或皮肤黏膜活动。在调整泼尼松剂量、抗疟药和免疫抑制剂使用情况后，居住国国内生产总值低、吸烟、关节炎、肾炎和补体水平低可预测疾病复发，而在随访时间≥50%处于低疾病活动状态（LLDAS50）是一个保护因素。肾脏活动和更高的时间调整后平均SLEDAI-2K可预测PAD，而LLDAS50具有保护作用。在验证队列中，所建立的模型对疾病复发和PAD的正确分类率分别为72.1%和83.8%。
疾病复发和PAD在SLE中都是常见的疾病活动模式；两者都可预测器官损害的累积，但在疾病特征和预测因素方面有所不同。由于9.7%的患者有PAD但未出现疾病复发，仅疾病复发指标不能充分涵盖所有疾病控制不佳的患者。
