Diagnosis of IgG4-Related Kidney Disease

  • Mitsuhiro KawanoEmail author
  • Kazunori Yamada


The diagnosis of IgG4-related disease (IgG4-RD) is usually based on the comprehensive diagnostic criteria proposed by a Japanese group in 2011 or consensus statement on the pathology of IgG4-RD published in 2012. In addition, two sets of organ specific criteria for IgG4-related kidney disease (IgG4-RKD) or IgG4-related tubulointerstitial nephritis are now available for the diagnosis of kidney lesions. These kidney-specific criteria sets are intented to complement the comprehensive diagnostic criteria. Although these criteria are useful in daily clinical practice for the early detection of IgG4-RKD, establishment and consensus of international classification criteria to cover all organs or tissues that may be involved are needed for selecting homogeneous groups of patients to better clarify the pathogenesis of the disease and its subtypes and to devise optimal strategies for their treatment.


Diagnostic criteria Classification criteria Diagnostic algorithm 



This work was supported in part by grants from Health and Labour Sciences Research Grants for the Study of Intractable Disease from Ministry of Health, Labor and Welfare, Japan.

We thank John Gelblum for his critical reading of the manuscript.




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Copyright information

© Springer Japan 2016

Authors and Affiliations

  1. 1.Division of Rheumatology, Department of Internal MedicineKanazawa University HospitalKanazawaJapan
  2. 2.Division of Rheumatology, Department of Internal MedicineKanazawa University HospitalKanazawaJapan

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