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Overview of IgG4-Related Kidney Disease

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IgG4-Related Kidney Disease

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Abstract

IgG4-related kidney disease (IgG4-RKD) is a comprehensive term for the renal lesions associated with IgG4-related disease (IgG4-RD), including IgG4-related tubulointerstitial nephritis (IgG4-related TIN), glomerular lesions, and IgG4-related pelvic lesion. The unifying disease entity of IgG4-RKD was elucidated after autoimmune pancreatitis and Mikulicz disease were recognized to occur frequently together. Involvement of multiple other organs was also identified to be part of this disease over the first dozen years of this century. A diagnostic algorithm and criteria for IgG4-RKD were proposed and published in 2011 by the IgG4-RKD working group of the Japanese Society of Nephrology. Simultaneously, another set of criteria for IgG4-related TIN was presented in the United States.

The proportion of IgG4-RKD in biopsy-proven cases is 0.67 % and its distribution does not show any regional differences in Japan. The most common histological feature of IgG4-RKD is tubulointerstitial nephritis, where the combination of IgG4-positive plasma cells and fiber bundles forms a characteristic fibrosis called “storiform” or “bird’s eye”. Glomerular lesions, most of which are membranous nephropathy, are observed in approximately 30 % of IgG4-RKD cases. In addition to the kidney parenchymal and pelvic lesions, tumefactive lesions can be located around the renal capsule and in the ureteral space and retroperitoneum. Accordingly, IgG4-RKD is closely associated with IgG4-related urological disease.

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Authors and Affiliations

  1. Sanko Clinic, 4-9-3 Ropponmatsu, Chuo-ku, Fukuoka, 810-0044, Japan

    Takao Saito M.D., Ph.D.

  2. Faculty of Medicine, Fukuoka University, Fukuoka, Japan

    Takao Saito M.D., Ph.D.

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  1. Takao Saito M.D., Ph.D.
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Correspondence to Takao Saito M.D., Ph.D. .

Editor information

Editors and Affiliations

  1. Faculty of Medicine, Fukuoka University, Fukuoka, Japan

    Takao Saito

  2. Rheumatology Clinic / Yawkey 2, Massachusetts General Hospital, Boston, Massachusetts, USA

    John H. Stone

  3. Department of Internal Medicine, Faculty of Medicine Fukuoka University, Fukuoka, Japan

    Hitoshi Nakashima

  4. Department of Internal Medicine, Nagaoka Red Cross Hospital, Nagaoka, Japan

    Takako Saeki

  5. Division of Rheumatology, Kanazawa University Hospital, Kanazawa, Japan

    Mitsuhiro Kawano

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Saito, T. (2016). Overview of IgG4-Related Kidney Disease. In: Saito, T., Stone, J., Nakashima, H., Saeki, T., Kawano, M. (eds) IgG4-Related Kidney Disease. Springer, Tokyo. https://doi.org/10.1007/978-4-431-55687-9_6

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  • DOI: https://doi.org/10.1007/978-4-431-55687-9_6

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  • Publisher Name: Springer, Tokyo

  • Print ISBN: 978-4-431-55686-2

  • Online ISBN: 978-4-431-55687-9

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