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Retroperitoneal Fibrosis and Arterial Lesions

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IgG4-Related Disease

Abstract

The condition of retroperitoneal fibrosis (RPF) has long been known, but since its inclusion in the spectrum of IgG4-RD, the disease concept requires reconsideration. Many cases of RPF correspond to periaortitis showing lesions around the abdominal aorta. Furthermore, similar lesions also occur in the thoracic aorta and aortic branches, and such lesions are now regarded as manifestations of periarteritis or frank arteritis. Unilateral RPF limited to one periureteral or perirenal pelvic area has been also sporadically reported. Marked lymphoplasmacytic infiltration and fibrosis are the characteristic pathological findings, with storiform fibrosis, obliterative phlebitis, and abundant IgG4-positive plasmacytic infiltration present. However, at this time it seems clear that IgG4-RD does not account for all cases of RPF. Some cases of RPF appear to have no association with IgG4-RD despite clinical and radiologic findings that are very similar to those of patients with IgG4-related RPF.

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Authors and Affiliations

  1. Department of Anatomic Pathology, Kurashiki Central Hospital, 1-1-1 Miwa, Kurashiki, Okayama, 710-8602, Japan

    Kenji Notohara

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  1. Kenji Notohara
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Correspondence to Kenji Notohara .

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Editors and Affiliations

  1. Department of Hematology and Immunology, Kanazawa Medical University, Ishikawa, Japan

    Hisanori Umehara

  2. The 3rd Department of Internal Medicine Div of Gastroenterology and Hepatology, Kansai Medical Univ Hirakata Hospital, Osaka, Osaka, Japan

    Kazuichi Okazaki

  3. Massachusetts General Hospital, Boston, Massachusetts, USA

    John H. Stone

  4. Shinshu University Center for Health Safety and Environment, Matsumoto, Nagano, Japan

    Shigeyuki Kawa

  5. Kanazawa University, Kanazawa, Ishikawa, Japan

    Mitsuhiro Kawano

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© 2014 Springer Japan

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Notohara, K. (2014). Retroperitoneal Fibrosis and Arterial Lesions. In: Umehara, H., Okazaki, K., Stone, J., Kawa, S., Kawano, M. (eds) IgG4-Related Disease. Springer, Tokyo. https://doi.org/10.1007/978-4-431-54228-5_26

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  • DOI: https://doi.org/10.1007/978-4-431-54228-5_26

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  • Publisher Name: Springer, Tokyo

  • Print ISBN: 978-4-431-54227-8

  • Online ISBN: 978-4-431-54228-5

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