TY - JOUR
T1 - Mediastinal-renal syndrome
T2 - A rare manifestation of granulomatosis with polyangiitis
AU - Okuezue, Beri A.
AU - Haqqie, Syed Shamim
AU - Foulke, Llewellyn A.
AU - Napier, Mark
AU - Vachharajani, Tushar
AU - Mathew, Roy O.
AU - Phelps, Kenneth
AU - Merrill, Donna
AU - Nayer, Ali
AU - Asif, Arif
N1 - Copyright:
Copyright 2013 Elsevier B.V., All rights reserved.
PY - 2012
Y1 - 2012
N2 - 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.
AB - 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.
KW - ANCA vasculitis
KW - Anti-proteinase 3 antibodies
KW - Granulomatosis with polyangiitis
KW - Mediastinal lymphadenopathy
KW - Mediastinal mass
KW - Pauci-immune crescentic glomerulonephritis
KW - Rapidly progressive glomerulonephritis
KW - Wegener's granulomatosis
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U2 - 10.2174/1874303X01306010020
DO - 10.2174/1874303X01306010020
M3 - Article
AN - SCOPUS:84876089234
VL - 6
SP - 20
EP - 25
JO - Open Urology and Nephrology Journal
JF - Open Urology and Nephrology Journal
SN - 1874-303X
IS - 1
ER -