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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 , *
Authors Info & Affiliations
Authors Info & Affiliations
The Open Urology & Nephrology Journal
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19 Mar 2013 •
RESEARCH ARTICLE
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DOI: 10.2174/1874303X01306010020
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..