Zhang Yuying, Qiao Lin, Zhang Liyun, Li Qin, Yang Pinting, Kong Xiaodan, Duan Xinwang, Zhang Miaojia, Li Xiaomei, Wang Yongfu, Xu Jian, Wang Yanhong, Hsieh Evelyn, Zhao Jiuliang, Xu Dong, Li Mengtao, Zhao Yan, Zeng Xiaofeng
Department of Rheumatology and Clinical Immunology, Chinese Academy of Medical Sciences & Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital (PUMCH), Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, 100730, China.
Department of Nuclear Medicine, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, China.
Clin Rheumatol. 2023 Feb;42(2):431-441. doi: 10.1007/s10067-022-06426-2. Epub 2022 Nov 16.
To investigate the clinical features and factors associated with primary Sjögren's syndrome (pSS)-associated renal tubular acidosis (RTA).
This case-control study was based on a multicenter pSS registry established by the Chinese Rheumatism Data Center. Patients with pSS, including those with RTA and those without renal involvement, between May 2016 and March 2020 were included in the analysis. Demographic, clinical, and laboratory data were also collected. Univariate and multivariate logistic regression analyses were used to identify factors that were associated with pSS-RTA.
This study included 257 pSS patients with RTA and 4222 patients without renal involvement. Significantly younger age at disease onset (40.1 ± 14.1 vs. 46.2 ± 13.1 years, P < 0.001), longer diagnosis interval (15.0 interquartile range [IQR] [1.0, 48.0] vs. 6.0 IQR [0, 34.0] months, P < 0.001), higher EULAR Sjögren's syndrome disease activity index (9 IQR [5, 15] vs. 3 IQR [0, 8], P < 0.001), and a higher prevalence of decreased estimated glomerular filtration rate (25.0% vs. 6.6%, P < 0.001) were observed in pSS patients with RTA than in those without renal involvement. Factors that were independently associated with pSS-RTA included age at disease onset ≤ 35 years (odds ratio [OR] 3.00, 95% confidence interval [CI] 2.27-3.97), thyroid disorders (OR 1.49, 95% CI 1.04-2.14), subjective dry mouth (OR 3.29, 95% CI 1.71-6.35), arthritis (OR 1.57, 95% CI 1.10-2.25), anti-SSB antibody positivity (OR 1.80, 95% CI 1.33-2.45), anemia (OR 1.67, 95% CI 1.26-2.21), elevated alkaline phosphatase level (OR 2.14, 95% CI 1.26-3.65), decreased albumin level (OR 1.61, 95% CI 1.00-2.60), and elevated erythrocyte sedimentation rate (OR 1.78, 95% CI 1.16-2.73).
Delayed diagnosis and decreased kidney function are common in pSS patients with RTA. pSS should be considered in patients with RTA, and early recognition and treatment may be useful in slowing the deterioration of renal function in patients with pSS-RTA. Key Points • pSS patients with RTA have earlier disease onset and higher disease activity than pSS patients without RTA, but the diagnosis was frequently delayed. • Decreased kidney function are common in pSS patients with RTA. • Sjögren's syndrome should be considered in young female patients with unexplained RTA, whereas RTA should be screened in pSS patients with early disease onset and elevated ALP level.
探讨原发性干燥综合征(pSS)相关肾小管酸中毒(RTA)的临床特征及相关因素。
本病例对照研究基于中国风湿病数据中心建立的多中心pSS登记系统。纳入2016年5月至2020年3月期间的pSS患者,包括合并RTA和无肾脏受累的患者。收集人口统计学、临床和实验室数据。采用单因素和多因素logistic回归分析确定与pSS-RTA相关的因素。
本研究纳入257例合并RTA的pSS患者和4222例无肾脏受累的患者。合并RTA的pSS患者发病年龄显著更小(40.1±14.1岁 vs. 46.2±13.1岁,P<0.001),诊断间隔更长(四分位数间距[IQR]为15.0[1.0, 48.0]个月 vs. 6.0[0, 34.0]个月,P<0.001),欧洲抗风湿病联盟(EULAR)干燥综合征疾病活动指数更高(9[IQR 5, 15] vs. 3[IQR 0, 8],P<0.001),估计肾小球滤过率降低的患病率更高(25.0% vs. 6.6%,P<0.001)。与pSS-RTA独立相关的因素包括发病年龄≤35岁(比值比[OR] 3.00,95%置信区间[CI] 2.27-3.97)、甲状腺疾病(OR 1.49,95% CI 1.04-2.14)、主观口干(OR 3.29,95% CI 1.71-6.35)、关节炎(OR 1.57,95% CI 1.10-2.25)、抗SSB抗体阳性(OR 1.80,95% CI 1.33-2.45)、贫血(OR 1.67,95% CI 1.26-2.21)、碱性磷酸酶水平升高(OR 2.14,95% CI 1.26-3.65)、白蛋白水平降低(OR 1.61,95% CI 1.00-2.60)和红细胞沉降率升高(OR 1.78,95% CI 1.16-2.73)。
合并RTA的pSS患者诊断延迟且肾功能下降常见。RTA患者应考虑pSS,早期识别和治疗可能有助于减缓pSS-RTA患者肾功能的恶化。要点 • 合并RTA的pSS患者比无RTA的pSS患者发病更早、疾病活动度更高,但诊断常延迟。 • 合并RTA的pSS患者肾功能下降常见。 • 对于不明原因RTA的年轻女性患者应考虑干燥综合征,而对于发病早且碱性磷酸酶水平升高的pSS患者应筛查RTA。