Mediastinal-Renal Syndrome: A Rare Manifestation of Granulomatosis with Polyangiitis

Okuezue , Beri A; Haqqie , Syed Shamim; Foulke , Llewellyn A; Napier , Mark; Vachharajani , Tushar; Mathew , Roy O; Phelps , Kenneth; Merrill , Donna; Nayer , Ali; Asif , Arif

Mediastinal-Renal Syndrome: A Rare Manifestation of Granulomatosis with Polyangiitis

Beri A Okuezue ¹, Syed Shamim Haqqie ¹, Llewellyn A Foulke ¹, Mark Napier ¹, Tushar Vachharajani ³, Roy O Mathew ¹, Kenneth Phelps ¹, Donna Merrill ¹, Ali Nayer ², Arif Asif ^{1, *}

¹ Division of Nephrology and Hypertension, Albany Medical College, Albany, NY, USA

² Division of Nephrology and Hypertension, University of Miami, Miami, FL, USA

³ Division of Nephrology and Hypertension, W.G. (Bill) Hefner Veterans Affairs Medical Center, Salisbury, NC, USA

Article Information

Identifiers and Pagination:

Year: 2013
Volume: 6
First Page: 20
Last Page: 25
Publisher ID: TOUNJ-6-20
DOI: 10.2174/1874303X01306010020

Article History:

Received Date: 7/1/2013
Revision Received Date: 19/2/2013
Acceptance Date: 20/2/2013
Electronic publication date: 19/3/2013
Collection year: 2013

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© Okuezue et al.; Licensee Bentham Open.

open-access license: This is an open access article licensed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.

^* Address correspondence to this author at the Division of Nephrology and Hypertension, Albany Medical College, 25 Hacket Blvd, Albany, NY 12208, USA; Tel: 518-262-0769; E-mail: asifa@mail.amc.edu

Granulomatosis with polyangiitis (GPA) is a distinct systemic disorder of obscure etiology characterized by necrotizing granulomatous inflammation and pauci-immune small-vessel vasculitis. Anti-neutrophil cytoplasmic antibodies (ANCA) appear to play an important role in the pathogenesis of GPA. Herein, we report a rare case of GPA presenting with ANCA-associated pauci-immune necrotizing and crescentic glomerulonephritis accompanied by a mediastinal mass and mediastinal lymphadenopathy in the absence of pulmonary parenchymal involvement. Furthermore, we describe the effects of plasmapheresis and concomitant immunosuppression on renal and mediastinal disease.

Keywords: Granulomatosis with polyangiitis, Wegener's granulomatosis, mediastinal mass, mediastinal lymphadenopathy, rapidly progressive glomerulonephritis, pauci-immune crescentic glomerulonephritis, ANCA vasculitis, anti-proteinase 3 antibodies..

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RESEARCH ARTICLE

Mediastinal-Renal Syndrome: A Rare Manifestation of Granulomatosis with Polyangiitis

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