目的:本研究旨在探讨原发性干燥综合征(pSS)患者雷诺现象(RP)的临床特征和危险因素。
方法:对333例新发pSS患者的病历进行回顾性分析。人口统计,临床,和血清学数据在有和没有RP的个体之间进行了比较。采用Logistic回归分析确定危险因素。
结果:11.41%的pSS患者存在RP。pSS-RP患者较年轻(49.74±14.56岁与54.46±13.20年,p=0.04),并表现出更高的疾病活动性(11[5.75-15]vs.7[4-12],p=0.03)比没有的。pSS伴RP患者肺部受累的患病率明显较高(60.53%vs.17.29%;p<0.001)。pSS-RP检测为抗核(ANA)阳性的患者比例明显更高,反RNP,和抗着丝粒抗体(ACA)相比,没有(分别为p=0.003,<0.001和0.01)。多变量分析确定了肺部受累(比值比[OR]=8.81,95%置信区间[CI]2.02-38.47;p=0.04),抗RNP阳性状态(OR=79.41,95%CI12.57-501.78;p<0.0001),以及ACA(OR=13.17,95%CI2.60-66.72;p=0.002)作为pSS-RP的预后因素。
结论:RP的存在定义了具有独特表型的pSS子集,表现为肺受累增加和抗RNP抗体和ACA的频率更高,以及更大的疾病活动。这些结果表明RP对pSS患者具有临床和预后价值。有必要对更多受试者进行进一步的前瞻性研究,以证实我们的发现并评估RP在pSS患者中的预后和治疗意义。关键点•333例原发性干燥综合征(pSS)患者中38例(11.41%)存在雷诺现象(RP),RP患者年龄较小,疾病活动度较高。•RP的存在表明具有独特表型的pSS子集,表现包括肺部受累增加以及抗RNP抗体和抗着丝粒抗体的出现频率更高。•pSS和RP患者需要密切随访和长期观察(包括评估微血管病变),特别关注系统性硬化症临床特征的可能发展。
OBJECTIVE: The aim at the current study was to investigate the clinical characteristics and risk factors of Raynaud\'s phenomenon (RP) in patients with primary Sjögren\'s syndrome (pSS).
METHODS: Retrospective analysis of the medical records of 333 new-onset pSS patients was performed. Demographic, clinical, and serological data were compared between individuals with and without RP. Logistic regression analysis was used to identify risk factors.
RESULTS: RP was present in 11.41% of the pSS patients. pSS-RP patients were younger (49.74±14.56 years vs. 54.46±13.20 years, p=0.04) and exhibited higher disease activity (11 [5.75-15] vs. 7 [4-12], p=0.03) than those without. The prevalence of lung involvement was significantly higher in pSS patients with RP (60.53% vs. 17.29%; p<0.001). A significantly higher proportion of patients with pSS-RP tested positive about antinuclear (ANA), anti-RNP, and anti-centromere antibodies (ACA) compared to those without (p=0.003, <0.001, and 0.01, respectively). Multivariate analysis identified lung involvement (odds ratio [OR]=8.81, 95% confidence interval [CI] 2.02-38.47; p=0.04), anti-RNP positive status (OR=79.41, 95% CI 12.57-501.78; p<0.0001), as well as ACA (OR=13.17, 95% CI 2.60-66.72; p=0.002) as prognostic factors for pSS-RP.
CONCLUSIONS: The presence of RP defined a subset of pSS with a unique phenotype, manifesting as increased lung involvement and a higher frequency of anti-RNP antibodies and ACA, as well as greater disease activity. These results suggest that RP has clinical and prognostic value of pSS patients. Further prospective studies with a larger number of subjects are warranted to confirm our findings and assess the prognostic and treatment implications of RP in pSS patients. Key Points • Raynaud\'s phenomenon (RP) was present in 38 (11.41%) of 333 patients with primary Sjögren\'s syndrome (pSS), with patients with RP exhibiting a younger age and higher disease activity. • The presence of RP indicates a subset of pSS with a unique phenotype, with manifestations including increased lung involvement and a higher frequency of anti-RNP antibodies and anti-centromere antibodies. • Patients with pSS and RP need close follow-up and long-term observation (including assessment of microangiopathy), with specific attention paid to the possible development of clinical features of systemic sclerosis.