Mediastinal-renal syndrome: A rare manifestation of granulomatosis with polyangiitis

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

doi:10.2174/1874303X01306010020

Mediastinal-renal syndrome: A rare manifestation of granulomatosis with polyangiitis

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

Medicine

Research output: Contribution to journal › Article › peer-review

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.

Original language	English (US)
Pages (from-to)	20-25
Number of pages	6
Journal	Open Urology and Nephrology Journal
Volume	6
Issue number	1
DOIs	https://doi.org/10.2174/1874303X01306010020
State	Published - 2012

Keywords

ANCA vasculitis
Anti-proteinase 3 antibodies
Granulomatosis with polyangiitis
Mediastinal lymphadenopathy
Mediastinal mass
Pauci-immune crescentic glomerulonephritis
Rapidly progressive glomerulonephritis
Wegener's granulomatosis

ASJC Scopus subject areas

Nephrology
Urology

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10.2174/1874303X01306010020

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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.

In: Open Urology and Nephrology Journal, Vol. 6, No. 1, 2012, p. 20-25.

Research output: Contribution to journal › Article › peer-review

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title = "Mediastinal-renal syndrome: A rare manifestation of granulomatosis with polyangiitis",

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 = "ANCA vasculitis, Anti-proteinase 3 antibodies, Granulomatosis with polyangiitis, Mediastinal lymphadenopathy, Mediastinal mass, Pauci-immune crescentic glomerulonephritis, Rapidly progressive glomerulonephritis, Wegener's granulomatosis",

author = "Okuezue, {Beri A.} and Haqqie, {Syed Shamim} and Foulke, {Llewellyn A.} and Mark Napier and Tushar Vachharajani and Mathew, {Roy O.} and Kenneth Phelps and Donna Merrill and Ali Nayer and Arif Asif",

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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

PY - 2012

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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.

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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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Mediastinal-renal syndrome: A rare manifestation of granulomatosis with polyangiitis

Abstract

Keywords

ASJC Scopus subject areas

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