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OA

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.