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Pathology of vasculitis: Wegener's granulomatosis, microscopic polyangiitis, renal limited vasculitis, and Churg–Strauss syndrome 

Pathology of vasculitis: Wegener's granulomatosis, microscopic polyangiitis, renal limited vasculitis, and Churg–Strauss syndrome
Chapter:
Pathology of vasculitis: Wegener's granulomatosis, microscopic polyangiitis, renal limited vasculitis, and Churg–Strauss syndrome
Author(s):

Franco Ferrario

and Maria Pia Rastaldi

DOI:
10.1093/med/9780199579655.003.0057
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date: 20 September 2020

1. ANCA-associated renal vasculitis, namely Wegener's granulomatosis, MPA, renal limited vasculitis and Churg–Strauss syndrome, display the same pathological features at renal biopsy. Only true interstitial granulomas are diagnostic of Wegener's granulomatosis.

2. Renal biopsy is a fundamental tool for diagnosis and management.

3. Glomerular and interstitial granulomas are acute lesions, very different from other kinds of chronic granulomatous reactions.

4. Despite the pauci-immune nature of the disease, a role for complement is emerging from more recent experimental and human studies.

5. Treatment options have largely improved prognosis, but relapses and adverse effects remain frequent and better therapies are still required.

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