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A case report of mixed P- and C-ANCA positive patients with pauci-immune crescentic glomerulonephritis
E Shimizu1, I Nakabayashi, T Kubota
1Second Department of Internal Medicine, National Defense Medical College, Saitama, Japan.
Insights
Two patients with pauci-immune crescentic glomerulonephritis and ANCA antibodies were studied. Their condition was diagnosed as microscopic polyarteritis nodosa or an undefined ANCA-associated vasculitis.
Area of Science:
- Nephrology
- Immunology
- Pulmonology
Background:
- Pauci-immune crescentic glomerulonephritis is a severe kidney disease often associated with anti-neutrophil cytoplasmic antibodies (ANCA).
- Cytoplasmic ANCA (C-ANCA) is typically linked to Wegener's granulomatosis, characterized by granulomatous inflammation.
Observation:
- Two patients presented with pauci-immune crescentic glomerulonephritis and elevated perinuclear ANCA (P-ANCA) and C-ANCA titers.
- Both patients exhibited pulmonary complications but lacked clinical or histological evidence of granulomatosis.
- Transbronchial lung biopsies revealed interstitial pneumonia and old hemorrhage, not Wegener's granulomatosis.
Findings:
- The patients' ANCA profiles, including reactivity to 29 and 59 kD bands on Western blots, were analyzed.
- Despite C-ANCA positivity, the absence of granulomatous disease distinguished these cases from typical Wegener's granulomatosis.
Implications:
- These cases suggest that ANCA-associated glomerulonephritis with pulmonary involvement can occur without granulomatous inflammation.
- The findings support the classification of such conditions as microscopic polyarteritis nodosa or a distinct ANCA-associated vasculitis.
- Further research is needed to fully delineate ANCA-associated vasculitides and their varied clinical presentations.
Abstract:
We describe 2 patients with pauci-immune crescentic glomerulonephritis and elevated titers of perinuclear and cytoplasmic anti-neutrophil cytoplasmic antibody (P-ANCA and C-ANCA) which were associated with both 29 and 59 kD bands on Western blots. The presence of C-ANCA corresponds with a clinical and/or histological diagnosis of Wegener's granulomatosis, but although both patients suffered from pulmonary complications there was no clinical nor histological evidence of granulomatosis. Transbronchial lung biopsy showed only interstitial pneumonia and old hemorrhage. Therefore, the patients' conditions were interpreted as microscopic polyarteritis nodosa or hitherto undefined vasculitis associated with ANCA.