What is Eosinophilic granulomatosis with polyangiitis?

Eosinophilic granulomatosis with polyangiitis is abbreviated as EGPA and can also be referred to as “Churg-Strauss Syndrome“, “Allergic Granulomatosis“, and “Allergic Angiitis“. Eosinophilic granulomatosis with polyangiitis is a defect resulting in the inflammation of the blood vessels in the system.  This case is detrimental as it impedes blood flow into certain tissues and organs. This obstruction of flow affects the organs and tissues badly as it can damage the system. Though, it is not contagious.  Picture Courtesy: emedicine Eosinophilic granulomatosis with polyangiitis is a rare case with minimal occurrences, though it is said to have no cure, and a common symptom indicating a possible presence of the EGPA is asthma. Other problems like rashes, pain, gastrointestinal bleeding, hay fever, and numbness can all be experienced. Allergic granulomatosis is also known to be an autoimmune disorder affecting blood vessels of small and medium-sized diameter in people majorly. Also, this occurs in people with previous cases of airway allergic hypersensitivity. Patients with the EGPA have heightened allergic-type blood cells known as eosinophils. Eosinophils are white blood cells that account for 5% or less of the whole white blood cell present in the body. When the Churg-Strauss syndrome is present, the eosinophils become responsible for more than 10% of the white blood cells in the body. The inflammation of the blood vessels is known as “Vasculitis“. Vasculitis results in unusual weakness of the vessels, and they tend to stretch. This case is known as an “Aneurysm“. The blood vessel walls become thinner, which can make them rupture, leading to leakage of blood into the tissues.  Picture Courtesy: emedicine Eosinophilic granulomatosis with polyangiitis affects the lungs, skin, joints, sinuses, nose, gastrointestinal tract, brain, kidney, or heart. EGPA affects all age groups ranging from children to adults. . Though the average age of diagnosis ranges from 35 to 50 years of age, it generally affects men and women. Therefore, it is not gender-specific.

What are the risk factors associated with EGPA?

 The risk factors that are associated with the EGPA are listed below:

• • • Asthma or airway problem:

Patients with previous cases of asthma, nasal allergies, or even chronic sinusitis are susceptible to the EGPA.

• • • Age

Based on statistics, persons within the age range of 35 to 50 years are liable to have the disorder.

What are the complications associated with EGPA?

Many organs are affected, such as the kidneys, heart, joints, lungs, gastrointestinal tract, etc. This disease can be fatal if not treated.  Complications that could occur vary depending on the affected organ. Complications are explained below:

• • • Skin Scarring

Sores that present scars on the skin result from the inflammation of the blood vessels.

• • • Kidney Damage

A disease known as glomerulonephritis can form in the kidney with the presence of the EGPA. It nulls the capabilities of the kidney to filter the waste products resulting in the accumulation of the products in the bloodstream.

• • • Heart Disease

When the heart is affected, the membrane encompassing the heart experiences inflammation alongside the muscular part of the heart, which results in a heart attack, further causing heart failure.

• • • Peripheral Nerve Damage

Damages to the nerves regulating movement in the legs and hand are imminent. Numbness and burning sensations are also experienced.

How is EGPA diagnosed?

The diagnosis of the EGPA can be made through the following:

• • • Physical Examination

During the physical examination, the doctor examines the patient thoroughly. By doing a physical examination, the doctor will be able to rule out possibilities of other diseases that appear similar to the EGPA.

• • • Medical History

History of nasal allergies, asthma, and other similar conditions associated with the EGPA can aid the diagnosis of the disease.

• • • Urinalysis

When a urine sample is taken, detection of excess protein or red blood cells in the urine sample indicates a possible EGPA. Also, the presence of blood in the urine.

• • • Blood Test

 A special test known as ANCA (Anti Neutrophilic Cytoplasmic Antibodies) test checks for abnormalities in blood count. They are present in almost 40% of the patients with EGPA. Other immunological tests such as elevated serum IgE levels, hypergammaglobulinemia, elevated levels of ECP (Eosinophilic Cationic protein), positive results for rheumatoid factor at low titer, etc. 

• • • Imaging Test

Computed tomography (CT) scan and X-rays can be used to check for irregularities in the sinuses and lungs. Other tests  ECG (Electrocardiogram) for cardiac manifestations GI endoscopy for GI bleeding EMG (Electromyography) and nerve conduction studies for peripheral neuropathies. Once the patient is suspected of having EGPA, to confirm the diagnosis, a biopsy is done by extracting a sample of the tissue to search for eosinophils or vasculitis.

Eosinophilic granulomatosis with polyangiitis Prevention

There is no specific cause for the EGPA. Therefore, there is no way of preventing the disease from occurring.

Eosinophilic granulomatosis with polyangiitis Treatment

Medications like immunosuppressive drugs that suppress the activation rate of the immune system are the best for the treatment of the EGPA. The drugs administered are based on the patients and affected organs. The drugs are:

• • • Glucocorticoids

These drugs include prednisone or prednisolone, used for patients with mild symptoms. 

• • • Corticosteroids

These drugs are used for patients with severe cases where the major organs like the heart and kidney are affected. This is combined with immunosuppressive drugs like cyclophosphamide, azathioprine methotrexate, or mycophenolate mofetil.

• • • Biologic Medications

One can use medications like rituximab and mepolizumab monoclonal antibodies to treat the EGPA. This is an alternative for people with mild symptoms where glucocorticoids are not effective enough.

What is the recovery rate of EGPA?

The duration of the intake of the medications administered can depend on the patient. Though, recovery of the patient ranges from a year to two years. The mortality rate without treatment is placed at 25% after 5 years.   

Symptoms

EGPA symptoms can vary from person to person. 

EGPA symptoms occur in three phases that may overlap.

• Prodromal

In this phase, the disease might be persistent over a long period whereby patients develop nasal polyposis, allergic rhinitis, asthma, or even a combination of a few symptoms.

• Second Phase

In this phase, the peripheral blood and tissue eosinophilia come to play, and the situation can almost be regarded as a Loffer syndrome which involves eosinophilic gastroenteritis and eosinophilic pneumonia.

• Third Phase

This phase is severe and life-threatening as the vasculitis (inflammation) develops and the organs begin to fail. Symptoms like fatigue, weight loss, e.t.c, are excessive in this state.

These phases may not occur serially, and the timing before each of them varies.

Other symptoms that may present themselves are:

• Fever.
• Hay fever.
• Fatigue.
• Loss of appetite.
• Joint pain.
• Pain in the muscle.
• Scars on the skin.
• Cough.
• Numbness.
• Pain in the abdomen.
• Bleeding in the gastrointestinal tract.
• Blood in stools and urine.
• Shortage of breath.

 

Causes

The specific cause of Eosinophilic granulomatosis with polyangiitis is not known. The human immune system contributes to the presence of EGPA. In an allergy mechanism where the tissues in the body are injured by the white blood cell, eosinophils’ products can also be responsible.

The major responsibility of the immune system is to fight invaders like germs, bacteria, and viruses responsible for diseases, infections, and other related problems. When this immune system becomes hyperactive, it can result in inflammation.

T lymphocytes in the body can be activated, therefore, increasing the inflammation of the vessels. Patients with a history of nasal polyposis, asthma, or allergic rhinitis are liable to have the EGPA.

Overview of EGPA

Picture Courtesy: Osmosis

FAQ

• • • How common is the Eosinophilic granulomatosis with polyangiitis?

The EGPA is a rare condition as it is said to occur in 3 people per 1 million people in a year.

• • • Who is affected by EGPA?

The EGPA can occur in anyone of any age range, but statistics show that the EGPA occurs in men and women between the ages of 35 to 50 years.

• • • Is EGPA a type of cancer or autoimmune disorder?

The eosinophilic granulomatosis with polyangiitis is not a type of cancer, and it is not associated with any autoimmune disorder.

• • • Does EGPA run in the family?

Even though the EGPA is associated with hereditary asthma, the EGPA can be passed through the genes.

• • • Are there side effects of treatment?

Immunosuppressive drugs can have side effects, so it is mandatory to visit the clinic for close monitoring regularly.