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RapidMLAMSRAGP

Eosinophilic granulomatosis with polyangiitis

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Cardiac, neurological or other organ-threatening EGPA

Myocarditis, heart failure, coronary vasculitis, mononeuritis multiplex, alveolar haemorrhage, glomerulonephritis, mesenteric ischaemia or severe eosinophilic lung disease requires emergency multidisciplinary treatment.

Action: Admit, obtain ECG, troponin, echocardiography and organ-directed imaging or biopsy, investigate infection and secondary eosinophilia, and start high-dose glucocorticoid plus rituximab or cyclophosphamide when organ-threatening vasculitis is credible.

Synopsis

Recognise eosinophilic granulomatosis with polyangiitis across asthma, eosinophilic tissue disease and vasculitis, exclude parasitic, drug and clonal eosinophilia, detect cardiac and neurological emergencies, and match induction and biologic treatment to organ severity.

  • Suspect EGPA when adult-onset asthma and chronic rhinosinusitis accompany eosinophilia, migratory lung infiltrates, purpura, neuropathy, cardiac, renal or gastrointestinal disease.
  • Check the absolute eosinophil count before high-dose steroid when safe, because glucocorticoid can normalise it rapidly and obscure phenotype.
  • MPO-ANCA supports a vasculitic subset but most patients can be ANCA-negative; never use a negative test to dismiss eosinophilic myocarditis or neuropathy.

Key red flags

Chest pain, syncope, arrhythmia, troponin rise, ventricular dysfunction or unexplained dyspnoea may indicate eosinophilic myocarditis even without striking CRP.

Mononeuritis multiplex

Acute painful asymmetric sensory loss and foot or wrist drop is a classic organ-threatening vasculitic feature.

Investigation priorities

01
FBC with absolute eosinophils and blood filmFirst step

Confirm eosinophilia before steroid and identify clonal or reactive haematological clues.

Management branches

First-line confirmationDefine eosinophilic, vasculitic and secondary causes

Asthma or sinus disease accompanies eosinophilia and systemic organ features.

  1. Record the pre-steroid eosinophil count, travel, medicines, atopy, asthma trajectory and skin, nerve, heart, lung, renal and gut findings.
  2. Send ANCA, urine, renal, cardiac and lung tests and a phenotype-directed parasite, fungal and clonal-eosinophilia screen.

Key medicines

PrednisoloneFor active systemic EGPA use 0.5–1 mg/kg orally once daily, with intravenous methylprednisolone 500 mg–1 g daily for three days in critical organ disease, followed by a planned taper.
BenralizumabFor adult relapsing or refractory EGPA use 30 mg subcutaneously once every four weeks as add-on treatment under NICE TA1096 and specialist supervision.
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Sources and review status7 sources · checked 27 Aug 2026 · clinical review pending
Sources

Sources and review status

National guidance is shown before implementation-dependent detail. Typical adult dose examples remain subject to patient factors, contraindications and the live BNF or specialist protocol. Source check completed 27 Aug 2026; clinical approval remains outstanding.

Authoring stateRapid draftClinical stateAwaiting reviewJurisdictionUnited Kingdom