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 | |
| 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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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