Department of Rheumatology and Immunology, Hebei General Hospital, No. 348 Heping West Road, Shijiazhuang, 050051, Hebei, China.
Department of Thoracic Surgery, Hebei General Hospital, Shijiazhuang, 050051, China.
Clin Rheumatol. 2022 Jul;41(7):2091-2102. doi: 10.1007/s10067-022-06135-w. Epub 2022 Mar 29.
The aim of the present study was to assess the clinical characteristic of hypocomplementemia (HC) in primary Sjogren's syndrome (pSS), and to address possible risk factors and the prognosis associated with HC in pSS patients.
pSS patients with HC in Hebei General Hospital from September 2016 to March 2019 were retrospectively analyzed and compared to those with normocomplementemia (NC). Logistic regression analysis was used to detect risk factors.
Of the 333 patients with pSS, 84 patients (25.23%) were presented with HC at diagnosis. The presence of hyper-IgG and anti-Ro52 antibodies was significantly more common in patients with HC. In addition to systemic involvement, pSS patients with HC had more hematological, renal, and nervous system involvement, and received more immunosuppressant treatments than NC group (p < 0.05). ESSDAI score was significantly higher in patients with HC (p < 0.05). Multivariate logistic analysis indicated that leukopenia (OR = 2.23) and hyper-IgG (OR = 2.13) were independent risk factors for pSS with HC. In addition, profound CD16/CD56+ NK-cell lymphopenia was found in pSS-HC patients. More pSS patients developed SLE in the HC group than NC group (4.76% vs. 0.80%, p = 0.04) during the follow-up.
HC was not an uncommon manifestation of pSS and had an independent association with the main clinical and immunological features. Patients with pSS-HC had an increased possibility to develop SLE that required more positive treatment with glucocorticoids and immunosuppressants.
• Hypocomplementemia had an independent association with the main clinical and immunological features in primary Sjogren's syndrome patients. • ESSDAI score was significantly higher in patients with hypocomplementemia. • The pSS patients with hypocomplementemia had an increased possibility to develop SLE.
本研究旨在评估原发性干燥综合征（pSS）患者低补体血症（HC）的临床特征，并探讨 pSS 患者 HC 相关的可能危险因素和预后。
回顾性分析 2016 年 9 月至 2019 年 3 月在河北医科大学第一医院就诊的 HC 的 pSS 患者，并与同期就诊的补体正常的 pSS 患者进行比较。采用 Logistic 回归分析检测危险因素。
在 333 例 pSS 患者中，84 例（25.23%）在诊断时出现 HC。HC 组患者高 IgG 和抗 Ro52 抗体阳性率显著高于 NC 组。除全身受累外，HC 组患者血液系统、肾脏和神经系统受累更为常见，接受免疫抑制剂治疗的患者也多于 NC 组（p<0.05）。HC 组患者 ESSDAI 评分显著高于 NC 组（p<0.05）。多因素 Logistic 分析表明白细胞减少（OR=2.23）和高 IgG（OR=2.13）是 pSS 合并 HC 的独立危险因素。此外，HC 组 pSS 患者存在显著的 CD16/CD56+NK 细胞淋巴细胞减少。在随访期间，HC 组发生系统性红斑狼疮（SLE）的患者多于 NC 组（4.76%比 0.80%，p=0.04）。
HC 并非 pSS 的罕见表现，与主要的临床和免疫学特征独立相关。HC 组 pSS 患者发生 SLE 的可能性增加，需要糖皮质激素和免疫抑制剂的积极治疗。
• 低补体血症与原发性干燥综合征患者的主要临床和免疫学特征独立相关。• 低补体血症患者的 ESSDAI 评分显著升高。• 低补体血症的原发性干燥综合征患者发生系统性红斑狼疮的可能性增加。
