Eosinophilic granulomatosis with polyangiitis

Author: Dr Mark Duffill, Dermatologist, Hamilton, New Zealand, 2008. Updated by A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand, January 2016.

What is eosinophilic granulomatosis with polyangiitis?

Eosinophilic granulomatosis with polyangiitis is a rare disease that affects the lungs, the skin and sometimes other organs. The name comes from the presence of eosinophils, granulomas and inflamed blood vessels. It is also known as Churg-Strauss syndrome, Churg-Strauss granulomatosis and allergic granulomatosis.

The main features of eosinophilic granulomatosis with polyangiitis are:

What are eosinophils?

Eosinophils are a type of white blood cell. They are often increased in number in allergic disorders.

What is the cause of eosinophilic granulomatosis with polyangiitis?

The cause of eosinophilic granulomatosis with polyangiitis is uncertain but the presence of asthma, eosinophilia and raised immunoglobulin E levels suggest an allergic process.

It has been associated with drug sensitivities to penicillin, penicillamine, iodides, leukotriene modifiers and mesalazine in some patients.

What are the clinical features of eosinophilic granulomatosis with polyangiitis?

Eosinophilic granulomatosis with polyangiitis has been divided into 3 distinct phases, which may or may not be sequential. Symptoms depend upon the phase and organs involved.

Prodromal phase
  • Respiratory symptoms dominate, especially asthma
  • Allergic rhinitis may also occur
Second phase
  • Peripheral blood eosinophilia
  • Eosinophilic tissue infiltration in the lungs and/or intestine
  • Weight loss, fever, sweats, abdominal pain and diarrhoea may occur
Vasculitic phase
  • May involve any organ: the heart is most frequently involved but the lung, kidney, lymph nodes and muscle can also be affected
  • Symptoms may include bloody diarrhoea, blood in the urine, joint aches, inflammation of the heart, convulsions, coma and damage to skin nerves
  • Asthma may lessen as the disease progresses
  • Skin involvement occurs in more than two thirds of patients:
    • Blisters, bleeding areas and nodules (lumps), often with central black dimple due to tissue breakdown (necrosis).
    • Affects backs of arms or fronts of legs, especially elbows and knees. Scalp or trunk may be involved.
    • Crops of new lesions may appear over months.

How is eosinophilic granulomatosis with polyangiitis diagnosed?

There is no specific blood test for eosinophilic granulomatosis with polyangiitis. Investigations usually include:

Imaging studies may include X-rays of the lungs and sinuses, and electrocardiogram (ECG).

Skin biopsy and/or kidney biopsy may demonstrate the diagnostic combination of tissue eosinophilia, leukocytoclastic vasculitis and granulomas.

What is the treatment for eosinophilic granulomatosis with polyangiitis?

Systemic corticosteroids are the first-line therapy for eosinophilic granulomatosis with polyangiitis. Mild disease may initially be treated with oral corticosteroids, but most authors suggest beginning therapy for extensive disease with intravenous (IV) corticosteroids. They are usually prescribed long-term.

The patient's response to corticosteroids is often dramatic. Within 1-2 weeks eosinophilia, the ESR and muscle enzyme levels may normalize. Corticosteroids can then be tapered, and remission is normally sustained. Low-dose prednisone may be continued for residual asthma or other symptoms.

If vasculitic symptoms are uncontrolled or if large doses of steroids are required, cyclophosphamide may be used. This appears to improve outcome in severe disease and may reduce the chance of relapse.

Other medications reported to be useful in eosinophilic granulomatosis with polyangiitis include:

Antibiotic prophylaxis with sulfamethoxazole-trimethoprim may be advised because intense immunosuppression increases the risk of infection.

What is the outcome for eosinophilic granulomatosis with polyangiitis?

Treatment of eosinophilic granulomatosis with polyangiitis with systemic steroids has improved the survival of patients with from 50% at 3 years to 75% at 8 years.

At least half of deaths in eosinophilic granulomatosis with polyangiitis are due to heart involvement.

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