ArticleKidney research and clinical practice2026
Clinical features and outcomes of immunoglobulin G4-related kidney disease and immunoglobulin G4-related retroperitoneal fibrosis in Korea.
Article in Kidney research and clinical practice, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
backgroundImmunoglobulin G4 (IgG4)-related disease (RD) is a newly recognized disease, and a few epidemiologic studies about this disorder have been published. This research aimed to demonstrate the clinical features and outcomes of IgG4-related kidney disease (RKD) and IgG4-related retroperitoneal fibrosis (RPF) compared to other organs' involvement.
methodsPatients who were diagnosed with IgG4-RD from January 2009 to July 2019 at three medical institutions in South Korea were included. They were classified into three groups: RKD, RPF, and Others groups. The differences in symptoms, laboratory, histological and radiological findings, treatment, and outcomes among the three groups were evaluated.
resultsOf 94 patients, 13 (13.8%) and 22 patients (23.4%) were classified into the RKD and RPF groups, respectively. There were older (p = 0.004) and more asymptomatic patients (p = 0.02) in the RKD and RPF groups. In the RKD group, hypocomplementemia (p = 0.003) and eosinophilia (p = 0.001) were more frequently identified. In logistic regression analysis, hypocomplementemia (odds ratio [OR], 14.04; 95% confidence interval [CI], 1.38-142.95) and decreased renal function at the time of diagnosis (OR, 0.95; 95% CI, 0.91-0.98) were associated with renal involvement. Older age (OR, 1.05; 95% CI, 1.00-1.11), male (OR, 6.11; 95% CI, 1.41-26.61), and higher serum IgG4 levels (OR, 1.00; 95% CI, 1.00-1.00) were associated with retroperitoneal involvement. The treatment duration was longer in the RKD and RPF groups (p = 0.01) with glucocorticoids.
conclusionRenal and retroperitoneal involvement in IgG4-RD presented clinical features that distinguish it from other organs' involvement, such as incidental diagnosis, hypocomplementemia, eosinophilia, and the need for a longer duration of maintenance treatment.
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