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Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)

A rare disease combining asthma, eosinophilia and systemic vasculitis.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dahiliye (İç Hastalıkları) department. Book Appointment →

What is Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)?

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly Churg-Strauss syndrome, is a rare disease that affects small- and medium-sized vessels and is characterised by asthma, peripheral eosinophilia and systemic vasculitis. Its annual incidence is around 1-3 per million.

EGPA classically progresses through three phases: a prodromal phase (allergic rhinitis and asthma), an eosinophilic phase (peripheral eosinophilia with organ infiltration) and a vasculitic phase (systemic vasculitis). Not every patient passes through all phases sequentially.

ANCA positivity (especially p-ANCA / anti-MPO) is found in 30-40% of cases. Cardiac involvement is the leading cause of death. Treatment relies on corticosteroids and other immunosuppressive agents.

Symptoms

Refractory asthma and allergic rhinitis
Peripheral eosinophilia (markedly raised eosinophil count)
Skin lesions (purpura, nodules)
Peripheral neuropathy (numbness, weakness)
Cardiac involvement (myocarditis, pericardial effusion)
Sinusitis and nasal polyposis
Renal involvement (glomerulonephritis)

Risk Factors

Long-standing severe asthma
Allergic rhinitis and nasal polyposis
Genetic predisposition (HLA association)
Use of leukotriene receptor antagonists (debated)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Peripheral eosinophilia and new organ involvement in an asthmatic patient
  • Unexplained peripheral neuropathy
  • Skin features of vasculitis (purpura, nodules)
  • Cardiac symptoms (chest pain, breathlessness, palpitations)

Treatment Methods

01
High-dose corticosteroid therapy (prednisolone)
02
Cyclophosphamide for severe organ involvement
03
Azathioprine or methotrexate for maintenance
04
Mepolizumab (anti-IL5 biological agent)
05
Urgent intensive treatment for cardiac involvement
06
Regular organ-function monitoring

Which Department to Visit?

You can visit our Dahiliye (İç Hastalıkları) department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.