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

Research output: Contribution to journalArticle

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 languageEnglish (US)
Pages (from-to)20-25
Number of pages6
JournalOpen Urology and Nephrology Journal
Volume6
Issue number1
DOIs
StatePublished - 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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  • Cite this

    Okuezue, B. A., Haqqie, S. S., Foulke, L. A., Napier, M., Vachharajani, T., Mathew, R. O., Phelps, K., Merrill, D., Nayer, A., & Asif, A. (2012). Mediastinal-renal syndrome: A rare manifestation of granulomatosis with polyangiitis. Open Urology and Nephrology Journal, 6(1), 20-25. https://doi.org/10.2174/1874303X01306010020