Chen Xu, Li Yanju, Chen Xi, Liu Yuanyuan, Xie Jing, Guo Donggeng
The Third Clinical Medicine College, Ningxia Medical University (People's Hospital of Ningxia Hui Autonomous Region), Yinchuan, 750002, China.
Key Laboratory of Autoimmune Diseases and Precision Medicie, People' s Hospital of Ningxia Hui Autonomous Region, Yinchuan, 750002, China.
Arch Dermatol Res. 2024 Dec 14;317(1):127. doi: 10.1007/s00403-024-03610-z.
Background though considered to have characteristics of systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) theoretically, Rhupus Syndrome (RS) owns its unique clinical features. In this retrospective cross-sectional study, we included 41 newly diagnosed Rhupus syndrome patients (NRSP). 160 new-diagnosed systemic lupus erythematosus patients (NSLEP) and 709 new-diagnosed rheumatoid arthritis patients (NRAP) were admitted as positive control groups. The clinical, serological, and radiological features among groups were compared, the disease activity of RA and SLE was evaluated, and the proportion of lymphocyte subsets in NRSPs under similar disease activity levels was explored. The initial onset age of RS patients was significantly younger than RA patients (P = 0.032), older than SLE patients (P = 0.008). 19.5% (8/41) RS patients initially presented with SLE symptom, while 12.2% RS patients presented with symptoms of both diseases. Hematopoietic dysfunction was the most prominent systemic manifestation in RS (61.0%, 25/41), only 9.8% patients experienced renal damage, and neurological disease were even rarer. Moreover, RS exhibited immunological characteristics different from NRAPs and NSLEPs, mainly manifested in decreased CD4 T cell and NK cell counts, increased ratio of CD8 + T (%) and total B cells, and decreased ratio of NK cells. RS is characterized by a higher incidence of interstitial lung disease and significant hyperglobulinemia besides the typical clinical characteristics of RA and SLE, which may be associated with a re-imbalanced lymphocyte subset. Evaluation of disease activity of RS cannot only rely on either SLEDAI-2 K or DAS28-ESR/-CRP, but more comprehensive assessment tools.
理论上,尽管狼疮性类风湿综合征(RS)被认为具有系统性红斑狼疮(SLE)和类风湿关节炎(RA)的特征,但其拥有独特的临床特点。在这项回顾性横断面研究中,我们纳入了41例新诊断的狼疮性类风湿综合征患者(NRSP)。160例新诊断的系统性红斑狼疮患者(NSLEP)和709例新诊断的类风湿关节炎患者(NRAP)作为阳性对照组。比较了各组的临床、血清学和放射学特征,评估了RA和SLE的疾病活动度,并探讨了在相似疾病活动水平下NRSP中淋巴细胞亚群的比例。RS患者的初始发病年龄显著低于RA患者(P = 0.032),高于SLE患者(P = 0.008)。19.5%(8/41)的RS患者最初表现为SLE症状,而12.2%的RS患者表现出两种疾病的症状。造血功能障碍是RS中最突出的全身表现(61.0%,25/41),仅有9.8%的患者出现肾损害,神经疾病则更为罕见。此外,RS表现出与NRAP和NSLEP不同的免疫学特征,主要表现为CD4 T细胞和NK细胞计数减少,CD8 + T(%)和总B细胞比例增加,以及NK细胞比例降低。除了RA和SLE的典型临床特征外,RS的特点是间质性肺疾病发病率较高和显著的高球蛋白血症,这可能与淋巴细胞亚群重新失衡有关。对RS疾病活动度的评估不能仅依赖于SLEDAI - 2K或DAS28 - ESR/-CRP,而需要更全面的评估工具。