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Publisher: Peytchinski Publishing
ISSN: 1312-773X (Online)
Issue: 2018, vol. 24, issue1
Subject Area: Medicine
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DOI: 10.5272/jimab.2018241.1872
Published online: 06 January 2018
Case report & Review article
J of IMAB. 2018 Jan-Mar;24(1):1872-1877
Immunoglobulin-g4(IgG4)-rich Pseudotumor of the Kidney – A case report and review of the literature
Orlin Savov1
, Abbas Agaimy2, Ralf Rieker2, Ekkehardt Bismarck1, Joachim Dörsam1, Georg C. Rass1, Thomas Ebert1, Arndt Hartmann2, Bernd J. Schmitz-Dräger1, 3,
1) Urologie24 and Department of Urology, St. Theresien-Krankenhaus Nürnberg, Germany
2) Institute of Pathology, Medical School, Philipp-Alexander-University Erlangen, Germany
3) Department of Urology, Philipp-Alexander-University, Erlangen, Germany.
ABSTRACT:
IgG4-related disease is a newly identified fibroinflammatory condition characterized by tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells and storiform fibrosis. Elevated serum IgG4 levels are frequently observed. IgG4-related disease affects predominantly middle-aged and elderly patients, with male predominance and responds favorably to steroids. The patients present with symptoms referable to the involvement of one or more sites either simultaneously or serially, usually in the form of mass lesions affecting various organs including pancreas, biliary tree, salivary glands, periorbital tissues, kidneys, lungs, retroperitoneum and lymph nodes. The case of a 61-year old male referred for therapy of a 6.6 cm tumorous lesion of the right kidney is presented. Histological assessment of the resected mass showed IgG4-rich sclerosing pseudotumor. The available literature is summarized, and diagnostic and therapeutic options for this condition are discussed. Consideration of a non-malignant disease may be reasonable even in cases of CAT scan findings suggestive of renal cancer.
Keywords: Interstitial nephritis, Immune complex, IgG4-related disease, Membranous glomerulonephritis, Membranous nephropathy, Renal mass,
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Please cite this article in PubMed Style or AMA (American Medical Association) Style:
Savov O, Agaimy A, Rieker R, Bismarck E, Dörsam J, Rass GC, Ebert T, Hartmann A, Schmitz-Dräger BJ. Immunoglobulin-g4(IgG4)-rich Pseudotumor of the Kidney – A case report and review of the literature. J of IMAB. 2018 Jan-Mar;24(1):1872-1877. DOI: 10.5272/jimab.2018241.1872
Correspondence to: Orlin Savov, Urologie24 and Department of Urology, St. Theresien-Krankenhaus Nürnberg; c/o Sulzbacher Str. 42, 90489 Nürnberg, Germany; E-mail: osavov@aol.com
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Received: 23 October 2017
Published online: 06 January 2018