Antineutrophil Cytoplasmic Autoantibody-negative Pauci-immune Necrotizing Glomerulonephritis with Plasma Cell-rich Tubulointerstitial Nephritis Complicated with Pleuritis and Digital Ischemia
- Resource Type
- Journal Article
- Authors
- Hajime Sanada; Kaori Yamaguchi; Taito Miyake; Takahiro Yamano
- Source
- Internal Medicine. 2024, 63(1):125
- Subject
- antineutrophil cytoplasmic autoantibody
digital ischemia
microscopic polyangiitis
pauci-immune necrotizing glomerulonephritis
pleuritis
- Language
- English
- ISSN
- 0918-2918
1349-7235
Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) predominantly affects small vessels. Almost all AAV patients are positive for myeloperoxidase- or proteinase 3-ANCA, and ANCA plays a crucial role in the pathogenesis of AAV. We herein report an ANCA-negative AAV patient with pauci-immune necrotizing glomerulonephritis and plasma cell-rich tubulointerstitial nephritis who was complicated with pleuritis and digital ischemia. ANCA-negative AAV is a rare clinical entity that is difficult to diagnose, and pleuritis and digital ischemia are rare manifestations of AAV. An early diagnosis and appropriate treatment are important, as any delay in the diagnosis may worsen the prognosis.