RESEARCH ARTICLE


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


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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


Abstract

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..