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, *
Identifiers and Pagination:Year: 2013
First Page: 20
Last Page: 25
Publisher Id: TOUNJ-6-20
Article History:Received Date: 7/1/2013
Revision Received Date: 19/2/2013
Acceptance Date: 20/2/2013
Electronic publication date: 19/3/2013
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.
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.