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Renal Lesions

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Abstract

Autoimmune pancreatitis (AIP) is currently divided into two distinct histological types – type 1 and type 2 – the former now being recognized as a condition representative of IgG4-related disease (IgG4-RD). IgG4-RD is a recently recognized emerging clinicopathological entity characterized by a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells with fibrosis and usually an elevated serum IgG4 concentration [1–3]. It is now clear that various inflammatory conditions affecting multiple organs fall within the category of IgG4-RD, including sclerosing cholangitis, sialadenitis, retroperitoneal fibrosis, and periaortitis, and that these have often been described as extra-pancreatic lesions associated with type 1 AIP. In the kidney, hydronephrosis due to retroperitoneal fibrosis was first reported as a renal lesion related to type 1 AIP [4], although it was considered to be a form of secondary renal injury caused by urinary tract obstruction. Renal parenchymal lesions associated with type 1 AIP were first described in case reports of tubulointerstitial nephritis (TIN) in 2004 [5, 6]. In fact, TIN is the most dominant feature associated with IgG4-RD in the kidney and is currently referred to as IgG4-related TIN [7, 8]. However, some glomerular lesions concurrent with IgG4-related TIN have also been reported, and several radiologically identified kidney lesions including pelvic lesions have been shown to be characteristic of IgG4-RD and useful for its diagnosis, in the setting of other forms of organ involvement that have been confirmed histopathologically [9, 10]. Therefore, the term “IgG4-related kidney disease” (IgG4-RKD) has recently been proposed as a comprehensive term for the renal lesions associated with IgG4-RD, including IgG4-related TIN, glomerular lesions concurrent with IgG4-related TIN, and IgG4-related pyelitis [9, 10]. In addition, glomerular lesions without IgG4-related TIN have also recently been reported in relation to type 1 AIP. This section focuses on whole renal lesions in type 1 AIP, including IgG4-RKD.

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Acknowledgments

This work was supported in part by grants from the Health and Labour Sciences Research Grants for the Study of Intractable Disease from Ministry of Health, Labor and Welfare, Japan, and “IgG4-Related Kidney Disease” working group of the Japanese Society of Nephrology.

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Authors and Affiliations

  1. Department of Internal Medicine, Nagaoka Red Cross Hospital, Senshu 2-297-1, Nagaoka, Niigata, 940-2085, Japan

    Takako Saeki MD, PhD

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  1. Takako Saeki MD, PhD
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Correspondence to Takako Saeki MD, PhD .

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Editors and Affiliations

  1. Department of Internal Medicine, Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan

    Terumi Kamisawa

  2. Department of Internal Medicine, Department of Internal Medicine, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea, Republic of (South Korea)

    Jae Bock Chung

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Saeki, T. (2015). Renal Lesions. In: Kamisawa, T., Chung, J. (eds) Autoimmune Pancreatitis. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-55086-7_18

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  • DOI: https://doi.org/10.1007/978-3-642-55086-7_18

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