Estimation of the number of histological diagnosis for IgG4-related kidney disease referred to the data obtained from the Japan Renal Biopsy Registry (J-RBR) questionnaire and cases reported in the Japanese Society of Nephrology Meetings

Clin Exp Nephrol. 2017 Feb;21(1):97-103. doi: 10.1007/s10157-016-1260-1. Epub 2016 Mar 25.


Background: More than 2 years have passed since the proposal of the diagnostic criteria for IgG4-related kidney disease (IgG4-RKD). The aim of this study was to estimate the number of histological diagnosis for IgG4-RKD throughout Japan and to clarify the regional distribution of the development of this disease.

Methods: A questionnaire was supplied to 140 research facilities registered in the Japan Renal Biopsy Registry (J-RBR). The items of the questionnaire were the total number of renal biopsies performed and the number of cases diagnosed as IgG4-RKD in 2012 and 2013 at each facility. Age, sex, and diagnosis category were also included for the IgG4-RKD cases. The geographic distribution of the disease development was evaluated using clinical case reports presented at the Eastern/Western regional meeting of the Japanese Society of Nephrology during the 15 years following 2001.

Results: Forty-seven facilities completed the questionnaire, resulting in a collection rate of 34 %. The total numbers of renal biopsies in 2012 and 2013 were 3387 and 3591, respectively. Forty-seven of these cases (24 in 2012 and 23 in 2013) were diagnosed as IgG4-RKD. The frequency of development of IgG4-RKD per one million over 40-year-old individuals during these 15 years varied between 0.9 and 3.1, depending on Japanese geographic region of Japan.

Conclusion: The results of the present survey indicate that the number of diagnosis for IgG4-RKD is approximately 130 cases per year throughout Japan, and no regional differences in disease frequency appear to exist.

Keywords: Geographic distribution; Histological diagnosis; IgG4-RKD.

Publication types

  • Multicenter Study

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Autoimmune Diseases / diagnosis*
  • Autoimmune Diseases / epidemiology*
  • Autoimmune Diseases / pathology
  • Biomarkers / analysis
  • Biopsy
  • Congresses as Topic
  • Female
  • Humans
  • Immunoglobulin G / analysis*
  • Japan / epidemiology
  • Kidney / immunology*
  • Kidney / pathology
  • Kidney Diseases / diagnosis*
  • Kidney Diseases / epidemiology*
  • Kidney Diseases / immunology
  • Male
  • Middle Aged
  • Registries
  • Retrospective Studies
  • Surveys and Questionnaires


  • Biomarkers
  • Immunoglobulin G