Eosinophilic granulomatosis with polyangiitis

What is it eosinophilic granulomatosis with polyangiitis?

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

The main characteristics of eosinophilic granulomatosis with polyangiitis are:

• Asthma
• Eosinophils infiltration In the lungs
• Eosinophils in the blood

• Small container vasculitis (inflamed blood vessels) and granulomas (mixed chronic inflammatory cells) in biopsy.

What are eosinophils?

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

What is the cause of eosinophilic granulomatosis with polyangiitis?

The cause of eosinophilic granulomatosis with polyangiitis is uncertain, but it is currently believed to be a autoimmune process.

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

What are the clinical characteristics of eosinophilic granulomatosis with polyangiitis?

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

How is eosinophilic granulomatosis with polyangiitis diagnosed?

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

• Complete blood count
• Rheumatoid factor (RhF), antinuclear antibody (ANA), antineutrofilo antibodies (HAUNCH)
• Kidney, liver and muscle function tests, as indicated by symptoms and signs.

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

Skin biopsy and kidney biopsy can demonstrate the diagnostic combination of tissue eosinophilia, vasculitis, and granulomas. See eosinophilic granulomatosis with polyangiitis pathology.

What is the treatment for eosinophilic granulomatosis with polyangiitis?

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

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

If vasculitic symptoms are not controlled or if large doses of steroids are required, cyclophosphamide can be used. This appears to improve outcome in severe disease and may reduce the chance of relapse.

Other medications reported useful in eosinophilic granulomatosis with polyangiitis include:

• Methotrexate
• Tumor necrosis factor (TNF) blockers, such as infliximab and etanercept
• Recombinant interferon (IFN) alpha
• Rituximab

Antibiotic prophylaxis Sulfamethoxazole-trimethoprim may be recommended because intense immunosuppression increases the risk of infection.

What is the result of eosinophilic granulomatosis with polyangiitis?

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

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