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18th February 2025
ANCA-associated vasculitides
It may be helpful to group some of the below conditions as investigation and management are similar. The primary ANCA-associated vasculitides are GPA, MPA, EGPA and IPIPC. Treatment, where there is life-/organ-threatening disease, is usually IV methylprednisolone and cyclophosphamide/rituximab.
| Upper airway and ENT features | Asthma | Nodules, cavities and infiltrates | Alveolar haemorrhage | c-ANCA positivity (%) | p-ANCA positivity (%) | |
|---|---|---|---|---|---|---|
| GPA | Very common | Rare | Very common | 5-10% | 70 | 25 |
| MPA | Rare | Rare | Uncommon | 10-30% | 25 | 60 |
| EGPA | Very common | Very common | Very common | Rare | < 5 | 35 |
| Anti-GBM | Rare | Rare | Very common | 25-60% | 5 | 25 |
Table 5 Typical pulmonary manifestations of several small-vessel vasculitides
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