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    Eosinophilic granulomatosis with polyangiitis

    Find symptoms and other information about Eosinophilic granulomatosis with polyangiitis.

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    Source : rarediseases.info.nih.gov

    EGPA: Symptoms, Causes & Treatment

    EGPA happens when certain types of cells in your blood or tissues inflame (swell). Those with this disease typically have a history of asthma and/or allergies.

    EGPA (formerly Churg-Strauss Syndrome)

    EGPA is a rare disease that happens when certain types of cells in your blood or tissues inflame (swell). The inflammation can affect many organs, including your heart and kidneys. Although the cause of the inflammation is unknown, experts suspect that it has something to do with the immune system failing to function correctly.

    OVERVIEW

    What is eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss Syndrome)?

    Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss Syndrome) is a disease caused by inflammation (swelling) that occurs in certain types of cells in your blood or in your tissues. Everyone who gets EGPA has a history of asthma and/or allergies. It can affect many of your organs.

    Almost all people with EGPA have increased numbers of "allergic type" blood cells called eosinophils. Eosinophils are a type of white blood cell that usually make up 5% or less of the total white blood cell count. In EGPA, eosinophils usually make up more than 10% of the total white blood cell count. In addition, most biopsies (tissue samples) contain clusters of cells called "granulomas" that may or may not involve blood vessels.

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    What is vasculitis?

    Vasculitis is a general medical term that means inflammation of the blood vessels. When your blood vessels become inflamed, several things can happen:

    They can grow weak and stretch, which can cause an aneurysm to develop. (An aneurysm is an abnormal blood-filled enlargement of a blood vessel.)

    The walls can become thinner. If they do, the walls can rupture, and blood can leak out into tissue.

    They can narrow to the point of closing. This can cause damage to the organs from the loss of oxygen and nutrients that were being supplied by the blood.

    What organs are affected by EGPA?

    This inflammation causes injury to your lungs, nose, sinuses, skin, joints, nerves, intestinal tract, heart and/or kidneys and rarely the brain.

    How common is EGPA?

    It is an extremely rare disease – there are only two to five new cases a year per one million people.

    Who is affected by EGPA?

    EGPA can occur in people of all ages, from children to the elderly. The average age that someone is diagnosed is between 35 and 50 years old. EGPA appears to affect men and women equally.

    Is EGPA contagious?

    No.

    SYMPTOMS AND CAUSES

    What causes eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss Syndrome)?

    The cause of EGPA inflammation is unknown.

    Your immune system plays a role in EGPA. The immune system acts to protect the body against “foreign invaders” (germs, bacteria) that cause infections, disease and other injuries to the body. Sometimes your immune system becomes over active and can cause inflammation.

    Is EGPA a type of cancer or autoimmune disorder?

    No.

    Does EGPA run in families?

    No, it’s not hereditary.

    What are EGPA symptoms?

    Because EGPA can affect several different organs, there is a wide range of symptoms.

    People who have EGPA may feel generally ill and fatigued or have fevers. They may lose their appetite and lose weight. Other symptoms depend on the organs or diseases involved. For example, you may have:

    Shortness of breath from asthma or inflammation in the air sacs and blood vessels of the lungs.

    Chest pain from disease that affects the lungs or heart.

    Rashes on the skin.

    Muscle and/or joint pain.

    Increased nasal discharge (runny nose) or facial pain from sinusitis.

    Abdominal pain or blood in the stools from intestinal tract involvement.

    Abnormal feelings, and numbness or loss of strength and feeling from nerve involvement.

    You may have any combination of these symptoms.

    Kidney disease caused by EGPA often does not have any symptoms. You may not know about inflammation of the kidney until the kidneys begin to stop working. Therefore, if you have any form of vasculitis, you must have regular urinalyses (examinations of the urine).

    DIAGNOSIS AND TESTS

    How is eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss Syndrome) diagnosed?

    The healthcare provider may use the following tests to diagnose EGPA:

    Medical history: to look for EGPA, especially asthma, allergies and other features of the disease.

    Physical examination: to discover which organs are involved and to rule out other illnesses that may look similar.

    Blood tests: to look for abnormal blood counts and an increase in eosinophils and special antibody testing called ANCA.

    Urinalysis: to detect whether there is too much protein, or red blood cells, in the urine.

    Imaging tests such as x-rays and computed tomography (CT) to look for any abnormalities in areas such as the lungs or sinuses.

    Once the diagnosis of EGPA is suspected, a biopsy (tissue sample) is often performed to try to find eosinophils, eosinophilic granulomas and/or vasculitis. Biopsies are not required in all cases, and are only recommended when abnormal findings are seen in the exam, laboratory tests, or imaging tests.

    MANAGEMENT AND TREATMENT

    Source : my.clevelandclinic.org

    Frontiers

    Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystemic disorder, belonging to the small vessel ANCA-associated vasculitis, defined as a eosinophil-rich and necrotizing granulomatous inflammation often involving the respiratory tract, and necrotizing vasculitis predominantly affecting small to medium-sized vessels, associated with asthma and eosinophilia. EGPA pathogenesis is not well known: HLA-DRB1*04 and *07, HLA-DRB4 and IL10.2 haplotype of the IL-10 promoter gene are the most studied genetic determinants. Among the acquired pathogenetic factors, the exposure to different allergens, infections, vaccinations, drugs and silica exposure have been involved. Eosinophils are the most characteristic cells in EGPA and different studies have demonstrated their role as effector and immunoregulatory cells. EGPA is considered a disease with a prevalent activation of the Th2 cellular-mediated inflammatory response but also humoral immunity plays an important role. A link between B and T inflammatory responses may explain different disease features. EGPA typically develops into three sequential phases: the allergic phase, distinguished by the occurrence of asthma, allergic rhinitis and sinusitis, the eosinophilic phase, in which the main pathological finding is the eosinophilic organ infiltrations (e.g. lungs, heart and gastrointestinal system) and the vasculitic phase, characterized by purpura, peripheral neuropathy and constitutional symptoms. ANCA (especially pANCA anti-MPO) are present in 40-60% of the patients. An elevation of IgG4 is frequently found. Corticosteroids and cyclophosphamide are classically used for remission induction, while azathioprine and methotrexate are the therapeutic options for remission maintenance. B-cell depletion with rituximab has shown promising results for remission induction.

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    Frontiers in Immunology

    Frontiers in Immunology Inflammation

    This article is part of the Research Topic

    Vascular inflammation in systemic autoimmunity View all 11 Articles

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    REVIEW article

    Front. Immunol., 03 November 2014 | https://doi.org/10.3389/fimmu.2014.00549

    Eosinophilic granulomatosis with polyangiitis: an overview

    Andrea Gioffredi*, Federica Maritati, Elena Oliva and Carlo Buzio

    Unit of Nephrology, University Hospital of Parma, Parma, Italy

    Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystemic disorder, belonging to the small vessel anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, defined as an eosinophil-rich and necrotizing granulomatous inflammation often involving the respiratory tract, and necrotizing vasculitis predominantly affecting small to medium-sized vessels, associated with asthma and eosinophilia. EGPA pathogenesis is not well known: * and * and IL10.2 haplotype of the IL-10 promoter gene are the most studied genetic determinants. Among the acquired pathogenetic factors, the exposure to different allergens, infections, vaccinations, drugs, and silica exposure have been involved. Eosinophils are the most characteristic cells in EGPA and different studies have demonstrated their role as effector and immunoregulatory cells. EGPA is considered as a disease with a prevalent activation of the Th-2 cellular-mediated inflammatory response and also humoral immunity plays an important role. A link between B and T inflammatory responses may explain different disease features. EGPA typically develops into three sequential phases: the allergic phase, distinguished by the occurrence of asthma, allergic rhinitis, and sinusitis, the eosinophilic phase, in which the main pathological finding is the eosinophilic organ infiltrations (e.g., lungs, heart, and gastrointestinal system), and the vasculitic phase, characterized by purpura, peripheral neuropathy, and constitutional symptoms. ANCA (especially pANCA anti-myeloperoxidase) are present in 40–60% of the patients. An elevation of IgG4 is frequently found. Corticosteroids and cyclophosphamide are classically used for remission induction, while azathioprine and methotrexate are the therapeutic options for remission maintenance. B-cell depletion with rituximab has shown promising results for remission induction.

    Introduction and Epidemiology

    Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystemic disorder, belonging to the small vessel anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs). According to the 1994 Chapel Hill consensus conference (CHCC), EGPA is defined as an eosinophil-rich and granulomatous inflammation often involving the respiratory tract, and necrotizing vasculitis predominantly affecting small to medium-sized vessels, associated with asthma and eosinophilia. Formerly known as “Churg–Strauss syndrome,” this eponym has been replaced during the 2012 Revised International CHCC, with the aim of focusing on the histopathology of the disease (1). Unlike in the 1990 American College of Rheumatology classification criteria and the former CHCC, the CHCC 2012 has reported for the first time that ANCA are found in EGPA, especially in patients with glomerulonephritis. This reflects some of the newest evidences of the distinction of two EGPA subsets, depending on the presence or the absence of ANCA (Table 1) (2).

    TABLE 1

    Table 1. Diagnostic criteria, classification, and nomenclature of eosinophilic granulomatosis with polyangiitis during the last 20 years.

    Eosinophilic granulomatosis with polyangiitis incidence in Europe is 0.5–6.8 new cases/year per million populations, whereas its prevalence is 10.7–13 cases per million populations. It mostly affects subjects between 40 and 60 years old and the mean age at diagnosis is 48 years (3).

    Pathogenesis

    Eosinophilic granulomatosis with polyangiitis pathogenesis is not well known. The disease is probably the result of a complex interaction in which genetically and environmental factors lead to an inflammatory response whose principal players are eosinophils, T, and B lymphocytes (2) (Figure 1).

    FIGURE 1

    Figure 1. Eosinophilic granulomatosis with polyangiitis pathogenesis.

    Genetic Determinants

    Eosinophilic granulomatosis with polyangiitis is an HLA-associated disease (4). It has been proven that it is associated with * and * (5) and with (6). This contraction of the class II HLA repertoire suggests a strong CD4+ T lymphocyte activation, possibly triggered by allergens or antigens.

    It has been also investigated the presence of single nucleotide polymorphisms (SNP) of the gene, which encodes interleukin (IL)-10, an important molecule for the activation of the Th-2 pathway; EGPA ANCA-negative subset has been associated with the IL10.2 haplotype of the IL-10 promoter gene, a condition, which leads to an increased production of IL-10 (7). This is apparently in line with EGPA pathogenesis, which is characterized by an increased Th-2 response and an increase in IgG4 levels, both of which seem to be mediated by IL-10.

    Source : www.frontiersin.org

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