原发干燥综合征相关肾脏范科尼综合征患者临床预后特点分析
Analysis of Clinical and Prognostic Characteristics in Patients with Primary Sjögren's Syndrome-Related Renal Fanconi Syndrome
摘要
Abstract
Objective Renal Fanconi syndrome(FS)is a rare renal manifestation of primary Sjögren's syndrome(pSS).This study aims to analyze the clinical and prognostic characteristics of patients with pSS-associ-ated renal FS(pSS-FS)and provide insights for clinical management.Methods Patients diagnosed with pSS-FS via renal biopsy at Peking Union Medical College Hospital from 1993 to 2024 were enrolled.Data collected in-cluded age,sex,clinical symptoms(xerostomia,xerophthalmia,skin purpura,arthralgia,polyuria,and systemic symptoms),laboratory findings[serum immunoglobulin G(IgG)and IgM,complement(C3,C4),antinuclear antibody,anti-Sjögren's syndrome-associated antigen A antibody(SSA),anti-SSB antibody,24-hour urinary pro-tein quantification,tubular proteinuria,serum creatinine,serum electrolytes],treatment,and follow-up informa-tion.Systematic assessments included the EULAR Sjögren's Syndrome Disease Activity Index(ESSDAI)score,pulmonary involvement(including non-infectious interstitial pneumonia,pulmonary fibrosis,pulmonary hyperten-sion,etc.),hematological involvement(anemia,leukopenia,thrombocytopenia),etc.Efficacy evaluations en-compassed improvements in immunological parameters,renal function,and tubular function.Group comparisons were performed using chi-square/Fisher's exact tests,t-tests,or non-parametric tests.Changes over time were an-alyzed using paired t-tests,and repeated measures during follow-up were assessed using mixed linear models.Results A total of 38 patients with pSS-FS were included,with 37(97.4%)being female.The median age at pSS diagnosis was 43(37,57)years.Xerostomia(76.3%)and xerophthalmia(71.1%)were the predominant clinical symptoms.The most common renal tubular dysfunctions were generalized aminoaciduria(96.9%),tubular proteinuria(96.0%),and hypokalemia(94.7%).The median eGFR was 52.57(32.04,76.10)mL/(min·1.73 m2),with60.5%(23/38)of patients having an eGFR below60 mL/(min·1.73 m2).After six months of immunosuppressive therapy,including moderate-to-high-dose glucocorticoids,significant improvements were observed in immunological parameters(improvement rate:69.2%),renal tubular function(89.5%),and renal function(44.4%).Following immunosuppressive treatment,the median eGFR increased from 54.95(33.06,76.10)mL/(min·1.73 m2)to 65.56(56.24,83.58)mL/(min·1.73 m2).Compared to patients with normal or mildly impaired baseline eGFR[≥60 mL/(min·1.73 m2)],those with significantly de-creased baseline eGFR[＜60 mL/(min·1.73 m2)]were older(46 years vs.37 years),had higher ESSDAI scores(16 vs.13),higher 24-hour urinary protein levels(1.16 g/d vs.0.48 g/d),but lower positivity rates of serum anti-SSA antibody(36.4%vs.86.7%)and anti-SSB antibody(22.7%vs.73.3%).Moreover,this group showed more pronounced renal function improvement after 6 months(58.8%vs.20.0%)and 12 months(66.7%vs.25.0%)of immunosuppressive therapy.Conclusions This study reports the clinical characteristics of the lar-gest single-center cohort of pSS-FS patients internationally,characterized by varying degrees of proximal renal tu-bular dysfunction and renal impairment.Timely initiation of immunosuppressive therapy,including glucocorticoids,is crucial,particularly for patients with significantly reduced eGFR,who may experience more substantial renal function improvement.关键词
原发干燥综合征/肾脏范科尼综合征/免疫抑制治疗/肾脏预后Key words
primary Sjögren syndrome/renal Fanconi syndrome/immunosuppressive therapy/renal prognosis分类
医药卫生引用本文复制引用
施潇潇,董源,姜嘉禾,夏鹏,张硕,文煜冰,徐东,张奉春,陈丽萌..原发干燥综合征相关肾脏范科尼综合征患者临床预后特点分析[J].协和医学杂志,2026,17(2):358-369,12.基金项目
国家自然科学基金(82570826,82200814) （82570826,82200814）
中央高水平医院临床科研专项(2025-PUMCH-A-161) （2025-PUMCH-A-161）
北京市自然科学基金(7252112) （7252112）
中国医学科学院医学与健康科技创新工程项目(CIFMS 2024-I2M-C&T-C-003) （CIFMS 2024-I2M-C&T-C-003）
中国医学科学院中央级公益性科研院所基本科研业务费(2023-PT320-10) （2023-PT320-10）
中央高校基本科研业务费(3332024206) （3332024206）
北京高层次创新创业人才支持计划"医疗卫生平台"领军人才项目(G202511003) National Natural Scientific Foundation of China(82570826,82200814) （G202511003）
National High Level Hospital Clinical Re-search Funding(2025-PUMCH-A-161) （2025-PUMCH-A-161）
Beijing Natural Science Foundation(7252112) （7252112）
CAMS Innovation Fund for Medical Sci-ences(CIFMS 2024-I2M-C&T-C-003) （CIFMS 2024-I2M-C&T-C-003）
Non-profit Central Research Institute Fund of Chinese Academy of Medical Sciences(2023-PT320-10) （2023-PT320-10）
Fundamental Research Funds for the Central Universities(3332024206) （3332024206）
Beijing High-Level Innovation and Entrepreneurship Talent Support Program-Healthcare Platform leading talent projects(G202511003) （G202511003）
