Eosinophilic granulomatosis with polyangiitis
Aka, Churg-Strauss
ANCA-associated disorder characterized by necrotizing granulomatous inflammation.
Manifests as both vascular and extravascular necrotizing granulomas with eosinophil blood and tissue infiltrations.
Three stages of eosinophilc granulomatosis with polyangiitis:
The
allergic stage is often marked by adult-onset
asthma, which can develop years before full presentation of the disorder.
Patients may also develop sinusitis, hemoptysis, and pulmonary infiltrates as part of the allergic stage.
The
eosinophilic stage is characterized by blood and tissue infiltration of eosinophils.
The
vasculitic stage can affect multiple organ systems:
– Nervous system involvement is associated with multiple mononeuropathy.
– Cutaneous involvement manifests as nodules or papules on the extensor surfaces, especially around the elbows.
– GI involvement can produce pain, diarrhea, and bleeding.
– Cardiac involvement often leads to inflammation and/or
cardiomyopathies.
Be aware that the kidneys are less commonly affected than in the other ANCA-associated vasculitides.
Disease onset is usually during the late 40s.
