Positive renal familial history in IgA nephropathy is associated with worse renal outcomes: a single-center longitudinal study

BMC Nephrology

Hiroyasu Tsukaguchi¹  Tran Thuy Huong Quynh¹  Tran Nguyen Truc Linh¹  Koichiro Higasa²  Fumihiko Koiwa³  Naoto Kawata³  Yoshinori Sato³  Kiyoko Inui³  Inoue Yoshihiko³  Ashio Yoshimura⁴ 

[1] 2nd Department of Internal Medicine, Kansai Medical University, Hirakata, Osaka 573-1010, Japan;Department of Genome Analysis, Institute of Biomedical Science, Kansai Medical University, Hirakata, Osaka 573-1010, Japan;Department of Internal Medicine, Division of Nephrology, Showa University School of Medicine, Fujigaoka Hospital, 1-30 Fujigaoka, Aobaku, 227-8501, Yokohama, Kanagawa, Japan;Department of Internal Medicine, Division of Nephrology, Showa University School of Medicine, Fujigaoka Hospital, 1-30 Fujigaoka, Aobaku, 227-8501, Yokohama, Kanagawa, Japan;Shinyokohama-Daiichi Clinic, Yokohama, Kanagawa, Japan;

关键词: IgA nephropathy;    End-stage renal disease;    Familial history;    Genetic factor;    Proteinuria;   

DOI  :  10.1186/s12882-021-02425-8

来源: Springer

【 摘 要 】

BackgroundIgA nephropathy (IgAN) is the most common primary glomerulonephritis worldwide. Although most IgAN cases are sporadic, few show a familial aggregation. However, the prevalence and prognosis of IgAN individuals with positive familial history (FH) of renal disorders remains uncertain. To address these issues, we conducted a longitudinal observational study on a single-institution cohort of patients with biopsy-proven IgAN.MethodsA total of 467 IgAN patients who underwent renal biopsy during 1994 to 2019 were ascertained to have positive- or negative-FH by history taking and were followed for an average of 8.9 years. We compared the clinical and pathological features of the two subgroups. The primary outcome, a composite of a hard endpoint (end-stage renal disease [ESRD]) and surrogate endpoint (a 50% or more reduction in the estimated glomerular filtration rate [eGFR] from baseline), was evaluated. To estimate the risk for progression to ESRD, a Cox proportional hazards analysis was performed for a subset of patients who underwent follow-up for > 2 years and had an eGFR > 30 mL/min/1.73 m2 at baseline (n = 389; observation, 8.7 years).ResultsPositive-FH subtype accounted for 11.6% (n = 54) of all IgAN patients. At baseline, there were no significant differences between the positive- and negative-FH subgroups regarding age, sex, comorbid disease, MEST-C score, observation period, and therapeutic interventions. However, the eGFR value at baselines was significantly lower in the positive-FH subgroup than in the negative-FH subgroup (P < 0.01). On multivariate analysis, positive-FH emerged an independent determinant of poorer renal outcomes (odds ratio, 2.31; 95% confidence interval, 1.10–4.85; P = 0.03), after adjusting for confounding factors. eGFR at follow-up was significantly lower in the positive-FH subgroup than in the negative-FH subgroup after adjustment for age and observation period.ConclusionsPositive-FH was found in 11.6% of all IgAN patients, consistent with the incidence seen in previous literature. A significantly lower eGFR at baseline and last follow-up and unfavorable renal outcomes in the positive-FH subgroup suggest that certain genetic risk factors predisposing to renal failure may exist in a fraction of our IgAN cohort. (331 words).

【 授权许可】

CC BY