Introduction
Eosinophilic granulomatosis with polyangiitis is a disorder affecting multiple systems and involving conditions like high levels of eosinophils (white blood cells which help to fight against the infection), small to medium-sized blood vessels necrotizing vasculitis, and extravascular granulomas. The inflamed vessels can affect various systemic organs like the lungs, gastrointestinal tract, skin, heart, and nerves. Churg-Strauss is a rare disorder with no treatment and has asthma in onset, with allergic rhinitis and nasal polyposis. It was first described by Jacob Churg and Lotte Strauss and the name suggested by Churg-Strauss after them; earlier, it was known as allergic granulomatosis.
What Are the Causes of Eosinophilic Granulomatosis With Polyangiitis?
The causes ofeosinophilic granulomatosis with polyangiitis are known. It is believed that the pathogenesis of the disorder has been genetically transmitted. The most researched genetic determinants include HLA-DRB1*04 and *07, HLA-DRB4, and the IL10.2 haplotype of the IL-10 promoter gene. Other causes like environmental factors involving allergens, medications, or an overreactive immune system.
What Are the Signs and Symptoms Associated With Eosinophilic Granulomatosis With Polyangiitis?
Eosinophilic granulomatosis with polyangiitis involves different organs and shows various signs and symptoms. -
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Loss of appetite.
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Joint and muscle pain.
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Abdominal pain and discomfort.
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Chest pain.
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Rashes.
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Blood in stool.
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Shortness of breath.
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Fatigue.
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Tingling over hand and legs.
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Sinusitis.
What Are the Stages Associated With Eosinophilic Granulomatosis With Polyangiitis?
Eosinophilic granulomatosis with polyangiitis has three phases associated with it.
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Prodromal is the first stage of eosinophilic granulomatosis with polyangiitis, which is marked by airway infections causing allergic rhinitis and asthma.
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The second stage follows the first stage leading to the excessive accumulation of eosinophils, causing tissue damage, especially tissues of the lungs and digestive tract.
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The third stage is the most dangerous stage and leads to vasculitis, causing cell death and life-threatening conditions.
What Is Vasculitis?
Vasculitis is a medical word used to describe inflammation of the blood vessels. Inflammation of blood vessels can lead to some serious conditions -
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Vasculitis can weaken and stretch the blood vessels, causing an aneurysm (an abnormal bulging or ballooning of a blood vessel's wall.).
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Vasculitis can also lead to the weakening and thinning of the walls of the blood vessels, which can lead to the leaking of the blood due to ruptured blood vessels.
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Vasculitis causes the narrowing of the vessels, which leads to decreased blood and oxygen supply to the other organs.
What Is the Epidemiology of Eosinophilic Granulomatosis With Polyangiitis?
Eosinophilic granulomatosis with polyangiitis is an extremely rare disease. There are only two or four cases detected over a year in one million people. However, it can be seen in children or adults. The commonly affected people are of the age group 35 to 50 years old, and the prevalence for the gender is equal.
In Eosinophilic Granulomatosis With Polyangiitis, How Many Organs Get Affected?
Eosinophilic granulomatosis with polyangiitis leads to the involvement of various organs and systems. The various systems involved, like renal, cardiac, or neurologic, indicate a poor prognosis.
The following are the organs involved -
Respiratory System -
Involvement of the respiratory system in eosinophilic granulomatosis with polyangiitis leads to asthma and is seen in the onset of the disease. Most patients tend to develop very serious conditions and become corticosteroid-dependent. Other respiratory problems like sinusitis are quite common and may vary from person to person. Though it is not destructive, it may lead to shortness of breath. Transient patchy pulmonary infiltrates are also very common.
Neurologic -
In eosinophilic granulomatosis with polyangiitis, neurologic manifestations are very common. Neurologic problems lead to multiple mononeuropathies (mono neuritis multiplex), which is seen in up to three-fourths of patients. Neurologic problems involve the central nervous system, which is rare but can include many problems, hemiparesis, confusion, seizures, and coma, with or without cranial nerve palsies or evidence of cerebral infarction.
Cutaneous -
In eosinophilic granulomatosis with polyangiitis involves, the skin is affected in about one-half of the affected individual. Nodules and papules appear on the extensor surfaces of extremities. Extravascular palisading granulomatous lesions cause central necrosis. Purpura or erythematous papules may develop due to leukocytoclastic vasculitis with or without prominent eosinophilic infiltration.
Musculoskeletal -
It involves the musculoskeletal system and causes arthralgias (joint pain), myalgias (muscle aches), or even arthritis. In addition, it leads to the weakening and thinning of the bones.
Cardiac -
Cardiac involvement in eosinophilic granulomatosis with polyangiitis is a major cause of mortality, resulting in heart failure due to myocarditis and endomyocardial fibrosis (inflammation of the heart muscle), coronary artery vasculitis, valvular disorders, and pericarditis. The predominant and important histopathologic finding is eosinophilic myocarditis. These are life-threatening conditions that need urgent medical attention.
Gastrointestinal -
Eosinophilic granulomatosis with polyangiitis causes gastrointestinal symptoms in one-third of the affected patients (For example, abdominal discomfort and pain, vomiting, diarrhea, bleeding, and acalculous cholecystitis) due to eosinophilic gastroenteritis (an inflammation of the lining of the stomach and intestines) or mesenteric ischemia (a condition due to a sudden decline in blood flow through the mesenteric vessels) caused by vasculitis.
Renal -
In vasculitis-associated disorders, the involvement of kidneys is less than in other disorders with antineutrophil cytoplasmic autoantibodies. Generally, disorders like pauci-immune (it is a type of immune complex), focal segmental necrotizing glomerulonephritis, and eosinophilic or granulomatous have rare involvement of kidneys.
What Is the Investigation Associated With the Diagnosis of Eosinophilic Granulomatosis With Polyangiitis?
Eosinophilic granulomatosis with polyangiitis can be diagnosed by following investigations -
Medical History:
To look for eosinophilic granulomatosis with polyangiitis, especially asthma, allergies, and other disease features.
Physical Examination:
Physical Examination is very important to examine the affected patient physically because it helps to identify the organs involved and to rule out other illnesses that may have similar signs and symptoms.
Physical examination involves inspection, auscultation, and palpitation.
Blood Tests:
Blood investigations are advised to determine abnormal blood counts, an increase in eosinophils, and special antibody testing called antineutrophil cytoplasmic antibodies (ANCA).
Urinalysis:
Urine investigations are performed to detect whether there is too much protein or red blood cells in the urine.
Imaging Test:
In eosinophilic granulomatosis with polyangiitis, imaging tests such as X-rays and computed tomography (CT) are performed to determine any abnormalities in areas such as the lungs or sinuses.
What Are the Treatment Modalities for the Treatment of Eosinophilic Granulomatosis With Polyangiitis?
Eosinophilic granulomatosis with polyangiitis is not a curable disease, but supportive treatment is given to the patient to avoid serious complications associated with the disease.
Supportive medical treatment involves -
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Corticosteroids.
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Other immunosuppressive drugs like - Azathioprine, Benralizumab, Cyclophosphamide, Methotrexate, and Rituximab.
Conclusion
Eosinophilic granulomatosis with polyangitis is a rare disorder that involves various organs of the body. The disease's prognosis is poor due to different internal organ involvement, which can worsen the condition. As the disease has no cure, it is recommended to consult an experienced healthcare provider for medical attention.
