Materiały źródłowe

• #1 Churg-Strauss syndrome – Symptoms & causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/churg-strauss-syndrome/symptoms-causes/syc-20353760

  Churg-Strauss syndrome is a disorder marked by blood vessel inflammation. […] The cause of Churg-Strauss syndrome is largely unknown. It’s likely that a combination of genes and environmental factors, such as allergens or certain medications, triggers an overactive immune system response. Instead of protecting against invading bacteria and viruses, the immune system targets healthy tissue, causing widespread inflammation. […] While anyone can get Churg-Strauss syndrome, people are usually around age 50 when diagnosed. Other potential risk factors include chronic asthma or nasal problems. Genetics and exposures to environmental allergens also may play a role.

• #2 EGPA (Formerly Churg-Strauss Syndrome): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/churg-strauss-syndrome-eosinophilic-granulomatosis-with-polyangiitis-egpa

  EGPA (formerly Churg-Strauss syndrome) begins with respiratory symptoms, but it can go on to affect you all over. […] EGPA is a rare disorder that occurs in people with a history of severe allergies and/or asthma. It affects your blood vessels, especially the ones in your lungs. […] Eosinophilic polyangiitis with granulomatosis (EGPA) develops from an overreaction from your immune system. Your immune system generates the inflammation in your blood vessels, the granulomas in your tissues and the eosinophilia that cause your symptoms. But researchers aren’t sure why this happens. […] Diseases that involve chronic inflammation from your immune system are sometimes called autoimmune diseases. One common feature of autoimmune diseases is that your body can develop antibodies that target certain cells in your body with inflammation. This may be one factor in EGPA.

• #3 Churg-Strauss Syndrome | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions/c/churg-strauss-syndrome.html

  Churg-Strauss syndrome is a rare disorder in which a person’s body builds up a large number of antibodies that normally fight bacteria, viruses or irritating or potentially dangerous substances. […] It is not yet know what causes this condition. Many researchers think that abnormal responses of the body’s immune system play a role in the disease. […] Many people who have Churg-Strauss Syndrome also have a history of allergies. […] Asthma or lung abnormalities sometimes occur a year or more before the more generalized, system-wide symptoms of Churg-Strauss appear.

• #4 Churg-Strauss syndrome: Symptoms, treatment, rash pictures

  https://www.medicalnewstoday.com/articles/churg-strauss-syndrome

  Churg-Strauss syndrome, or eosinophilic granulomatosis with polyangiitis (EGPA), is a rare condition that causes vasculitis, meaning it restricts blood flow. This restriction of blood flow affects multiple organs, especially the lungs. […] Research has not yet identified a specific cause for Churg-Strauss syndrome, but the following types of factors likely play a role: genetic, immunological, environmental. […] Though the triggers may be unclear, scientists believe the condition occurs as an autoimmune disease, meaning that the immune system attacks healthy cells. […] More studies are necessary to determine the exact cause of Churg-Strauss syndrome, but many researchers believe genetics play a role in the condition.

• #5 Churg-Strauss Syndrome Symptoms, Causes, and Treatment

  https://www.verywellhealth.com/churg-strauss-sydrome-4174962

  Churg-Strauss syndrome, also called eosinophilic granulomatosis with polyangiitis (EGPA), is a rare autoimmune condition that causes inflammation of blood vessels (vasculitis). […] The exact cause of Churg-Strauss is unknown. Like other autoimmune disorders, researchers believe that several factors, such as genetics and environment, play a role in „triggering” the immune system. […] Between 39% and 59% of people diagnosed with Churg-Strauss syndrome also have detectable levels of antineutrophil cytoplasmic antibodies (ANCA). […] The use of Accolate (zafirlukast) has been associated with the development of Churg-Strauss. This drug is a leukotriene antagonist used to treat asthma, and research on other drugs in this class such as Singulair (montelukast) also found a relationship. It’s unclear if it’s the drugs themselves that cause Churg-Strauss syndrome.

• #6 Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome) – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK537099/

  In recent years, efforts have been made to subclassify clinical phenotypes of EGPA based on the presence or absence of vasculitis, which is somewhat counterintuitive to the nomenclature itself. […] The primary trigger in the pathogenesis at the cellular level appears to be an aberrant T-helper (Th)-cell pathway. […] Various theories have been proposed regarding what triggers the abnormal TH2-type immune response in EGPA. Allergies, infections, and medications have all been considered potential factors. […] The pathogenic role of T lymphocytes in this aberrant immune response has been supported by the presence of specific clonally expanded T-cell subpopulations and an increased frequency of related HLA alleles, such as HLA-DRB1:04 and HLA-DRB1:07. […] The presence of ANCA alone is not an absolute indicator of vasculitis. In one study, about 47% of patients had vasculitis without seropositivity for ANCA, while 29% tested positive for myeloperoxidase (MPO) ANCA without evidence of vasculitis.

• #7 Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome) | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/19543

  In recent years, efforts have been made to subclassify clinical phenotypes of EGPA based on the presence or absence of vasculitis, which is somewhat counterintuitive to the nomenclature itself. […] Patients who are ANCA-positive (approximately 30%-40% of all EGPA patients) typically exhibit a vasculitic phenotype, often presenting with myalgia, migrating polyarthralgia, weight loss, mononeuritis multiplex, and renal involvement, such as crescentic or necrotizing glomerulonephritis. […] Conversely, patients who are ANCA-negative tend to have an eosinophilic phenotype with a higher incidence of myocarditis. […] The primary trigger in the pathogenesis at the cellular level appears to be an aberrant T-helper (Th)-cell pathway. […] Various theories have been proposed regarding what triggers the abnormal TH2-type immune response in EGPA.

• #8 Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome) – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK537099/

  Patients who are ANCA-positive (approximately 30%-40% of all EGPA patients) typically exhibit a vasculitic phenotype, often presenting with myalgia, migrating polyarthralgia, weight loss, mononeuritis multiplex, and renal involvement, such as crescentic or necrotizing glomerulonephritis. […] Conversely, patients who are ANCA-negative tend to have an eosinophilic phenotype with a higher incidence of myocarditis.

• #9 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/333492-overview

  The majority of EGPA cases are idiopathic and are attributed to a complex interplay of polygenic risk factors and environmental exposures. Genetic analysis in a small population of EGPA patients found an association with HLA-DRB 1*04 and HLA-DRB 1*07, whereas HLA-DRB 1*03 and HLA-DRB1*13 proved to be protective. […] A genome-wide association study (GWAS) by Lyons et al demonstrated genetic differences between myeloperoxidase (MPO)-ANCApositive EGPA compared with ANCA-negative EGPA, suggesting that those may be two distinct disease entities. […] MPO-ANCApositive EGPA typically has vasculitic features, such as neuropathy and glomerulonephritis, and is strongly associated with the HLA-DQ genes, which are responsible for encoding the cell surface protein receptors found on antigen-presenting cells.

• #10 Eosinophilic Granulomatosis with Polyangiitis – Apfed

  https://apfed.org/about-ead/eosinophilic-granulomatosis-with-polyangiitis/

  Eosinophilic Granulomatosis with Polyangiitis (EGPA), formerly known as Churg-Strauss Syndrome, is a rare autoimmune disorder characterized by inflammation of blood vessels and the presence of high numbers of white blood cells known as eosinophils. […] The cause of EGPA is not known, although it is believed to result from an interaction of genetics and the environment, complicated by an overactive immune system. […] Because all patients with EGPA have high levels of eosinophils at some point during their disease, it is thought that there may be some dysregulation of eosinophil production, maturation, or development. […] Anti-neutrophil cytoplasmic antibodies (ANCA) are also present in many patients with EGPA and may also play a role in EGPA disease development. […] While the exact role of ANCA in EGPA and other vasculitides remains unclear, it is felt that the binding of ANCA to vascular walls contributes to vascular inflammation and injury as well as chemotaxis of inflammatory cells.

• #11 Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome) | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/19543

  Allergies, infections, and medications have all been considered potential factors. […] The pathogenic role of T lymphocytes in this aberrant immune response has been supported by the presence of specific clonally expanded T-cell subpopulations and an increased frequency of related HLA alleles, such as HLA-DRB1:04 and HLA-DRB1:07. […] In addition, an increased serum presence of interleukin 10 (IL-10) exists, which mediates the inhibition of TH1 response, thus favoring the differentiation of TH2 cells.

• #12 EGPA (Formerly Churg-Strauss Syndrome): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/churg-strauss-syndrome-eosinophilic-granulomatosis-with-polyangiitis-egpa

  Researchers have grouped EGPA together with a handful of similar conditions that all involve a certain autoimmune antibody, called ANCA. They call these conditions ANCA-associated vasculitis, or AAV. However, only about 40% of people with EGPA have the ANCA antibody, so its not the only factor. […] Eosinophilia seems to be a separate cause of symptoms that often occur long before vasculitis does. When eosinophils accumulate in your tissues, they trigger inflammation and damage them. Eosinophilia is related to asthma and allergies. People with asthma are 34 times more at risk of EGPA than others.

• #13 Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome) – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK537099/

  In recent years, efforts have been made to subclassify clinical phenotypes of EGPA based on the presence or absence of vasculitis, which is somewhat counterintuitive to the nomenclature itself. […] The primary trigger in the pathogenesis at the cellular level appears to be an aberrant T-helper (Th)-cell pathway. […] Various theories have been proposed regarding what triggers the abnormal TH2-type immune response in EGPA. Allergies, infections, and medications have all been considered potential factors. […] The pathogenic role of T lymphocytes in this aberrant immune response has been supported by the presence of specific clonally expanded T-cell subpopulations and an increased frequency of related HLA alleles, such as HLA-DRB1:04 and HLA-DRB1:07. […] The presence of ANCA alone is not an absolute indicator of vasculitis. In one study, about 47% of patients had vasculitis without seropositivity for ANCA, while 29% tested positive for myeloperoxidase (MPO) ANCA without evidence of vasculitis.

• #14 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/333492-overview

  The majority of EGPA cases are idiopathic and are attributed to a complex interplay of polygenic risk factors and environmental exposures. Genetic analysis in a small population of EGPA patients found an association with HLA-DRB 1*04 and HLA-DRB 1*07, whereas HLA-DRB 1*03 and HLA-DRB1*13 proved to be protective. […] A genome-wide association study (GWAS) by Lyons et al demonstrated genetic differences between myeloperoxidase (MPO)-ANCApositive EGPA compared with ANCA-negative EGPA, suggesting that those may be two distinct disease entities. […] MPO-ANCApositive EGPA typically has vasculitic features, such as neuropathy and glomerulonephritis, and is strongly associated with the HLA-DQ genes, which are responsible for encoding the cell surface protein receptors found on antigen-presenting cells.

• #15 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/333492-overview

  The majority of EGPA cases are idiopathic and are attributed to a complex interplay of polygenic risk factors and environmental exposures. Genetic analysis in a small population of EGPA patients found an association with HLA-DRB 1*04 and HLA-DRB 1*07, whereas HLA-DRB 1*03 and HLA-DRB1*13 proved to be protective. […] A genome-wide association study (GWAS) by Lyons et al demonstrated genetic differences between myeloperoxidase (MPO)-ANCApositive EGPA compared with ANCA-negative EGPA, suggesting that those may be two distinct disease entities. […] MPO-ANCApositive EGPA typically has vasculitic features, such as neuropathy and glomerulonephritis, and is strongly associated with the HLA-DQ genes, which are responsible for encoding the cell surface protein receptors found on antigen-presenting cells.

• #16 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/333492-overview

  In contrast, ANCA-negative EGPA has a very different genetic signature. It is less strongly associated with HLA-DQ but rather has an increased presentation of glycoprotein A33 (GPA33) and thymic stromal lymphopoietin (TSLP). […] Research has identified several environmental risk factors for EGPA. Maritati et al evaluated environmental exposures in 111 patients with EGPA and reported that the odds ratio of farming exposure in EGPA was 2.68, and exposure to silica had an odds ratio of 3.26. […] There are a few documented cases of massive inhalational exposures to harvest grain dust, flour, and pigeon droppings resulting in EGPA. […] A few cases of drug-induced EGPA have been reported. These have included mesalazine-induced EGPA in a patient with Crohn disease and sclerosing cholangitis, clarithromycin-induced EGPA in a patient with chronic pneumonia, and dupilumab-induced EGPA in a patient with refractory asthma. […] An EGPA-like syndrome is a rare complication that develops in steroid-dependent patients with asthma who have their oral steroid dose reduced after they start treatment with a leukotriene receptor antagonist.

• #17 Eosinophilic Granulomatosis with Polyangiitis, formerly Churg-Strauss Syndrome (EGPA) : Johns Hopkins Vasculitis Center

  https://www.hopkinsvasculitis.org/types-vasculitis/churgstrauss-syndrome-css/

  The cause of EGPA is unknown but is probably multi-factorial. Genetics may play a small role in the disease, but EGPA is almost never seen in two members of the same family. Environmental factors such as exposure to industrial solvents may play a role in susceptibility to this disease, but this is largely speculative. Infections may be the inciting event(s), but to date there is no definitive evidence of this.

• #18 Churg Strauss Syndrome – Vasculitis UK

  https://www.vasculitis.org.uk/about-vasculitis/churg-strauss-syndrome

  Eosinophilic Granulomatosis with Polyangiitis (EGPA), previously known as Churg-Strauss Syndrome, is an inflammatory disease of small and medium sized blood vessels. […] The cause of EGPA is not known. It is likely that a combination of factors lead to development of EGPA. Studies looking at genetics have shown some genes that are linked to EGPA, while some people have Anti Neutrophil Cytoplasmic Antibodies (ANCA) which may cause some types of auto-immune disease. Unusual levels of some types of hormone-like chemicals in the blood (cytokines) have also been found in people with EGPA, and this may contribute too.

• #19 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/333492-overview

  In contrast, ANCA-negative EGPA has a very different genetic signature. It is less strongly associated with HLA-DQ but rather has an increased presentation of glycoprotein A33 (GPA33) and thymic stromal lymphopoietin (TSLP). […] Research has identified several environmental risk factors for EGPA. Maritati et al evaluated environmental exposures in 111 patients with EGPA and reported that the odds ratio of farming exposure in EGPA was 2.68, and exposure to silica had an odds ratio of 3.26. […] There are a few documented cases of massive inhalational exposures to harvest grain dust, flour, and pigeon droppings resulting in EGPA. […] A few cases of drug-induced EGPA have been reported. These have included mesalazine-induced EGPA in a patient with Crohn disease and sclerosing cholangitis, clarithromycin-induced EGPA in a patient with chronic pneumonia, and dupilumab-induced EGPA in a patient with refractory asthma. […] An EGPA-like syndrome is a rare complication that develops in steroid-dependent patients with asthma who have their oral steroid dose reduced after they start treatment with a leukotriene receptor antagonist.

• #20 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/333492-overview

  In contrast, ANCA-negative EGPA has a very different genetic signature. It is less strongly associated with HLA-DQ but rather has an increased presentation of glycoprotein A33 (GPA33) and thymic stromal lymphopoietin (TSLP). […] Research has identified several environmental risk factors for EGPA. Maritati et al evaluated environmental exposures in 111 patients with EGPA and reported that the odds ratio of farming exposure in EGPA was 2.68, and exposure to silica had an odds ratio of 3.26. […] There are a few documented cases of massive inhalational exposures to harvest grain dust, flour, and pigeon droppings resulting in EGPA. […] A few cases of drug-induced EGPA have been reported. These have included mesalazine-induced EGPA in a patient with Crohn disease and sclerosing cholangitis, clarithromycin-induced EGPA in a patient with chronic pneumonia, and dupilumab-induced EGPA in a patient with refractory asthma. […] An EGPA-like syndrome is a rare complication that develops in steroid-dependent patients with asthma who have their oral steroid dose reduced after they start treatment with a leukotriene receptor antagonist.

• #21 Churg-Strauss Disease: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1178795-overview

  Churg-Strauss syndrome or disease (CSD), now known as eosinophilic granulomatosis with polyangiitis (EGPA), is a specific variant of the group of diseases characterized by necrotizing vasculitis of small- and medium-sized systemic blood vessels. […] Although these conditions are not thought to be directly infectious, microbial superantigens may play a role in provoking the onset of the dysregulated immune response that gives rise to these conditions. […] Several lines of evidence suggest a genetic predisposition to Churg-Strauss disease (CSD), now known as eosinophilic granulomatosis with polyangiitis (EGPA), which may entail an inherited tendency to dysregulation of the cellular immune system. […] Environmental factors may contribute to the development of EGPA. For example, the inhalation of fungal spores, such as those produced by actinomycetes and Aspergillus species, has been implicated in the pathogenesis of some cases.

• #22 Churg-Strauss Disease: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1178795-overview

  Churg-Strauss syndrome or disease (CSD), now known as eosinophilic granulomatosis with polyangiitis (EGPA), is a specific variant of the group of diseases characterized by necrotizing vasculitis of small- and medium-sized systemic blood vessels. […] Although these conditions are not thought to be directly infectious, microbial superantigens may play a role in provoking the onset of the dysregulated immune response that gives rise to these conditions. […] Several lines of evidence suggest a genetic predisposition to Churg-Strauss disease (CSD), now known as eosinophilic granulomatosis with polyangiitis (EGPA), which may entail an inherited tendency to dysregulation of the cellular immune system. […] Environmental factors may contribute to the development of EGPA. For example, the inhalation of fungal spores, such as those produced by actinomycetes and Aspergillus species, has been implicated in the pathogenesis of some cases.

• #23 Eosinophilic Granulomatosis with Polyangiitis (EGPA) – Brigham and Women’s Hospital

  https://www.brighamandwomens.org/lung-center/diseases-and-conditions/eosinophilic-granulomatosis-with-polyangiitis

  A rare systemic vasculitis affecting less than two out of every million people each year, eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a condition that causes inflammation of small-sized blood vessels in people with asthma. […] The cause of EGPA is unknown. Potential risk factors may include: […] Asthma, allergic rhinitis and/or nasal polyps […] Drug sensitivities to penicillin, penicillamine, iodides, leukotriene modifiers or mesalazine […] Environmental exposure to inhaled allergens such as silica dust.

• #24 Eosinophilic Granulomatosis with Polyangiitis, formerly Churg-Strauss Syndrome (EGPA) : Johns Hopkins Vasculitis Center

  https://www.hopkinsvasculitis.org/types-vasculitis/churgstrauss-syndrome-css/

  The cause of EGPA is unknown but is probably multi-factorial. Genetics may play a small role in the disease, but EGPA is almost never seen in two members of the same family. Environmental factors such as exposure to industrial solvents may play a role in susceptibility to this disease, but this is largely speculative. Infections may be the inciting event(s), but to date there is no definitive evidence of this.

• #25 What Is Churg-Strauss Syndrome?

  https://www.icliniq.com/articles/immune-disorders/churg-strauss-syndrome

  Churg-Strauss syndrome is an autoimmune disorder wherein there is inflammation of small and medium-sized blood vessels (vasculitis or angiitis), especially of the skin, lungs, abdomen, and nerves. […] In Churg-Strauss syndrome, there is an exaggerated and abnormal immune system activity to some unclear triggers (medications, infections, etc.). The exact cause is not clear, but multiple factors are speculated to cause this syndrome. […] Exposure to industrial solvents. […] Infections. […] Autoimmunity. […] Medications (Zafirlukast therapy). […] Genetics (least suspected cause).

• #26 Churg-Strauss Disease: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1178795-overview

  Carbamazepine, macrolide antibiotics, and cysteinyl leukotriene-receptor antagonists have been implicated as provocative causes of EGPA. […] Leukotriene-receptor antagonists may be especially important in terms of provoking chronic EGPA that does not resolve with discontinuation of the inciting drug.

• #27 Churg-Strauss Syndrome Symptoms, Causes, and Treatment

  https://www.verywellhealth.com/churg-strauss-sydrome-4174962

  Churg-Strauss syndrome, also called eosinophilic granulomatosis with polyangiitis (EGPA), is a rare autoimmune condition that causes inflammation of blood vessels (vasculitis). […] The exact cause of Churg-Strauss is unknown. Like other autoimmune disorders, researchers believe that several factors, such as genetics and environment, play a role in „triggering” the immune system. […] Between 39% and 59% of people diagnosed with Churg-Strauss syndrome also have detectable levels of antineutrophil cytoplasmic antibodies (ANCA). […] The use of Accolate (zafirlukast) has been associated with the development of Churg-Strauss. This drug is a leukotriene antagonist used to treat asthma, and research on other drugs in this class such as Singulair (montelukast) also found a relationship. It’s unclear if it’s the drugs themselves that cause Churg-Strauss syndrome.

• #28 What Is Eosinophilic Granulomatosis with Polyangiitis: Symptoms, Causes, Diagnosis, Treatments

  https://www.webmd.com/lung/egpa

  EGPA is an extremely rare disease that causes inflammation of your blood vessels. It can damage small and medium-sized blood vessels. Doctors also call it Churg-Strauss vasculitis, eosinophilic granulomatosis with polyangiitis (EGPA), and allergic angiitis. […] Doctors dont know exactly what causes it. Some experts think allergies play a role. But many people with allergies never get it. Others say its a mix of genes and things in your environment, like allergens and medications, that send your immune system into overdrive. Instead of protecting you, it ramps up an immune response that causes inflammation throughout your body. […] Theres no proven tie to medications. And while its rare, some people did get it after they used an asthma medication called montelukast. Others got it when they switched from oral steroids for allergies and asthma to inhaled versions.

• #29 Churg-Strauss Disease: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1178795-overview

  Carbamazepine, macrolide antibiotics, and cysteinyl leukotriene-receptor antagonists have been implicated as provocative causes of EGPA. […] Leukotriene-receptor antagonists may be especially important in terms of provoking chronic EGPA that does not resolve with discontinuation of the inciting drug.

• #30 Churg-Strauss Disease: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1178795-overview

  Carbamazepine, macrolide antibiotics, and cysteinyl leukotriene-receptor antagonists have been implicated as provocative causes of EGPA. […] Leukotriene-receptor antagonists may be especially important in terms of provoking chronic EGPA that does not resolve with discontinuation of the inciting drug.

• #31 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/333492-overview

  In contrast, ANCA-negative EGPA has a very different genetic signature. It is less strongly associated with HLA-DQ but rather has an increased presentation of glycoprotein A33 (GPA33) and thymic stromal lymphopoietin (TSLP). […] Research has identified several environmental risk factors for EGPA. Maritati et al evaluated environmental exposures in 111 patients with EGPA and reported that the odds ratio of farming exposure in EGPA was 2.68, and exposure to silica had an odds ratio of 3.26. […] There are a few documented cases of massive inhalational exposures to harvest grain dust, flour, and pigeon droppings resulting in EGPA. […] A few cases of drug-induced EGPA have been reported. These have included mesalazine-induced EGPA in a patient with Crohn disease and sclerosing cholangitis, clarithromycin-induced EGPA in a patient with chronic pneumonia, and dupilumab-induced EGPA in a patient with refractory asthma. […] An EGPA-like syndrome is a rare complication that develops in steroid-dependent patients with asthma who have their oral steroid dose reduced after they start treatment with a leukotriene receptor antagonist.

• #32 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/333492-overview

  In contrast, ANCA-negative EGPA has a very different genetic signature. It is less strongly associated with HLA-DQ but rather has an increased presentation of glycoprotein A33 (GPA33) and thymic stromal lymphopoietin (TSLP). […] Research has identified several environmental risk factors for EGPA. Maritati et al evaluated environmental exposures in 111 patients with EGPA and reported that the odds ratio of farming exposure in EGPA was 2.68, and exposure to silica had an odds ratio of 3.26. […] There are a few documented cases of massive inhalational exposures to harvest grain dust, flour, and pigeon droppings resulting in EGPA. […] A few cases of drug-induced EGPA have been reported. These have included mesalazine-induced EGPA in a patient with Crohn disease and sclerosing cholangitis, clarithromycin-induced EGPA in a patient with chronic pneumonia, and dupilumab-induced EGPA in a patient with refractory asthma. […] An EGPA-like syndrome is a rare complication that develops in steroid-dependent patients with asthma who have their oral steroid dose reduced after they start treatment with a leukotriene receptor antagonist.

• #33 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/333492-overview

  In contrast, ANCA-negative EGPA has a very different genetic signature. It is less strongly associated with HLA-DQ but rather has an increased presentation of glycoprotein A33 (GPA33) and thymic stromal lymphopoietin (TSLP). […] Research has identified several environmental risk factors for EGPA. Maritati et al evaluated environmental exposures in 111 patients with EGPA and reported that the odds ratio of farming exposure in EGPA was 2.68, and exposure to silica had an odds ratio of 3.26. […] There are a few documented cases of massive inhalational exposures to harvest grain dust, flour, and pigeon droppings resulting in EGPA. […] A few cases of drug-induced EGPA have been reported. These have included mesalazine-induced EGPA in a patient with Crohn disease and sclerosing cholangitis, clarithromycin-induced EGPA in a patient with chronic pneumonia, and dupilumab-induced EGPA in a patient with refractory asthma. […] An EGPA-like syndrome is a rare complication that develops in steroid-dependent patients with asthma who have their oral steroid dose reduced after they start treatment with a leukotriene receptor antagonist.

• #34 Churg–Strauss syndrome: Case series | Pulmonology

  https://www.journalpulmonology.org/en-churgstrauss-syndrome-case-series-articulo-S2173511512000061

  ChurgStrauss syndrome (CSS) is a systemic necrotizing vasculitis of the small and medium vessels, associated with extravascular eosinophilic granulomas, peripheral eosinophilia and asthma. […] This is a rare syndrome of unknown etiology, affecting both genders and all age groups. […] The etiology of CSS is unknown. Proposed hypotheses include an auto-immune process, possibly associated with genetic and environmental factors. […] In the last decade, the leukotriene receptor antagonists (LTRAs), including zafirlukast and montelukast have been reported to be associated with the development of CSS. […] This is probably related to steroid withdrawal after starting LTRAs in patients with hitherto attenuated CSS or the prescription of LTRAs to patients with worsening asthma which was actually unrecognized worsening CSS.

• #35 What Are the Signs and Symptoms of Churg-Strauss Syndrome?

  https://www.emedicinehealth.com/what_are_the_symptoms_of_churg-strauss_syndrome/article_em.htm

  Churg-Strauss syndrome is the traditional name for eosinophilic granulomatosis with polyangiitis (EGPA), a rare rheumatologic disease that affects multiple organ systems because it causes inflammation in the small blood vessels (vasculitis) throughout the body. This can also result in asthma and blood cell abnormalities. […] The cause of Churg-Strauss (eosinophilic granulomatosis with polyangiitis syndrome) is unknown. It is thought to be a result of an immune dysfunction or an autoimmune reaction, in which the body mistakenly attacks its own tissues. It is unclear what causes the autoimmune reaction, as to whether it is a type of allergic reaction or if it is triggered by the environment or certain medications. […] Genetic factors may also play a role. […] Some medications that are thought to be associated with Churg-Strauss syndrome include the following: Leukotriene modifying agents, Glucocorticoids, Omalizumab, Cocaine.

• #36 Churg–Strauss syndrome: Case series | Pulmonology

  https://www.journalpulmonology.org/en-churgstrauss-syndrome-case-series-articulo-S2173511512000061

  ChurgStrauss syndrome (CSS) is a systemic necrotizing vasculitis of the small and medium vessels, associated with extravascular eosinophilic granulomas, peripheral eosinophilia and asthma. […] This is a rare syndrome of unknown etiology, affecting both genders and all age groups. […] The etiology of CSS is unknown. Proposed hypotheses include an auto-immune process, possibly associated with genetic and environmental factors. […] In the last decade, the leukotriene receptor antagonists (LTRAs), including zafirlukast and montelukast have been reported to be associated with the development of CSS. […] This is probably related to steroid withdrawal after starting LTRAs in patients with hitherto attenuated CSS or the prescription of LTRAs to patients with worsening asthma which was actually unrecognized worsening CSS.

• #37 Churg–Strauss syndrome: Case series | Pulmonology

  https://www.journalpulmonology.org/en-churgstrauss-syndrome-case-series-articulo-S2173511512000061

  In fact there have been reports of CSS after starting inhaled steroids and omalizumab and a recent case crossover study showed that several asthma control medications are associated with the onset of CSS, supporting the hypothesis that an aggravation of asthma is associated with CSS onset, rather than a specific drug.

• #38 Recent Advances in the Diagnosis of Churg-Strauss Syndrome | Modern Pathology

  https://www.nature.com/articles/3880475

  The absence of necrosis in the vessel wall in no way mitigates against the diagnosis. […] CSS granulomas consist of necrotic eosinophils and Charcot-Leyden crystals, usually with a surrounding palisade of giant cells or epithelioid histiocytes. […] Granulomas are not required for the diagnosis of CSS, and the absence of granulomas should not influence the pathologist against a diagnosis of CSS. […] In many cases, CSS responds well to steroid therapy. However, steroids are also widely used to treat asthma itself. Thus some patients develop CSS, but the disease is more or less completely, and accidentally, suppressed by steroid therapy for the underlying asthma, only to appear in clinically recognizable form if steroid therapy is discontinued or manipulated. […] This sequence of events has been the source of some debate in the literature.

• #39 Recent Advances in the Diagnosis of Churg-Strauss Syndrome | Modern Pathology

  https://www.nature.com/articles/3880475

  Over the past few years, leukotriene receptor antagonist (LRA) therapy for asthma has been introduced; the two most commonly used drugs, zafirlukast and montelukast, are selective antagonists of the Type 1 cysteinyl leukotriene receptor. […] Because most new asthma therapies are likely to be steroid sparing, it is likely that further cases will appear as each agent is introduced, and the pathologist should be aware of this phenomenon.

• #40 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/333492-overview

  In contrast, ANCA-negative EGPA has a very different genetic signature. It is less strongly associated with HLA-DQ but rather has an increased presentation of glycoprotein A33 (GPA33) and thymic stromal lymphopoietin (TSLP). […] Research has identified several environmental risk factors for EGPA. Maritati et al evaluated environmental exposures in 111 patients with EGPA and reported that the odds ratio of farming exposure in EGPA was 2.68, and exposure to silica had an odds ratio of 3.26. […] There are a few documented cases of massive inhalational exposures to harvest grain dust, flour, and pigeon droppings resulting in EGPA. […] A few cases of drug-induced EGPA have been reported. These have included mesalazine-induced EGPA in a patient with Crohn disease and sclerosing cholangitis, clarithromycin-induced EGPA in a patient with chronic pneumonia, and dupilumab-induced EGPA in a patient with refractory asthma. […] An EGPA-like syndrome is a rare complication that develops in steroid-dependent patients with asthma who have their oral steroid dose reduced after they start treatment with a leukotriene receptor antagonist.

• #41 Churg-Strauss Syndrome: Symptoms, Causes, and Diagnosis

  https://www.healthline.com/health/churg-strauss-syndrome

  Its not clear what causes Churg-Strauss syndrome. However, asthma seems to be a common denominator in people with the condition. […] There have been studies that have looked at whether or not one of the ingredients in a common medication for severe asthma, montelukast, may cause or trigger it. […] So far, there is not enough evidence to show that montelukast causes Churg-Strauss. However, there is evidence indicating that montelukast may trigger Churg-Strauss if Its already a previously undetected condition. […] Its known that Churg-Strauss syndrome is not genetic and is not contagious. Its also known that an autoimmune condition is involved in some way in the cause of this condition.

• #42 Churg-Strauss Syndrome | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions/c/churg-strauss-syndrome.html

  Churg-Strauss syndrome is a rare disorder in which a person’s body builds up a large number of antibodies that normally fight bacteria, viruses or irritating or potentially dangerous substances. […] It is not yet know what causes this condition. Many researchers think that abnormal responses of the body’s immune system play a role in the disease. […] Many people who have Churg-Strauss Syndrome also have a history of allergies. […] Asthma or lung abnormalities sometimes occur a year or more before the more generalized, system-wide symptoms of Churg-Strauss appear.

• #43 Churg-Strauss syndrome » Global Autoimmune Institute

  https://www.autoimmuneinstitute.org/autoimmune-resources/autoimmune-diseases-list/churg-strauss-syndrome/

  EGPA, or Churg-Strauss Syndrome, is the inflammation of blood vessels (vasculitis), clustering of white blood cells, and development of granulomas (inflammatory nodular lesions). […] A history of asthma or nasal allergies is the main risk factor for developing EGPA, and typically precedes EGPA onset by 5 to 9 years.

• #44 Eosinophilic Granulomatosis with Polyangiitis (EGPA) – Brigham and Women’s Hospital

  https://www.brighamandwomens.org/lung-center/diseases-and-conditions/eosinophilic-granulomatosis-with-polyangiitis

  A rare systemic vasculitis affecting less than two out of every million people each year, eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a condition that causes inflammation of small-sized blood vessels in people with asthma. […] The cause of EGPA is unknown. Potential risk factors may include: […] Asthma, allergic rhinitis and/or nasal polyps […] Drug sensitivities to penicillin, penicillamine, iodides, leukotriene modifiers or mesalazine […] Environmental exposure to inhaled allergens such as silica dust.

• #45 Churg-Strauss Syndrome: Causes, Symptoms, Diagnosis & Treatment

  https://www.medindia.net/health/conditions/churg-strauss-syndrome.htm

  Churg-Strauss syndrome is a condition characterized by blood vessel inflammation that can restrict blood flow to vital organs and tissues. In some cases it can lead to permanent damage of these organs. […] Churg-Strauss syndrome is usually diagnosed in people between ages 38 and 52. It is almost absent in children and older people. Another risk factor for Churg-Strauss syndrome is having a history of severe nasal allergies, chronic sinusitis or asthma. […] Churg-Strauss syndrome is a rare disease and the number of people with the syndrome has not been clearly estimated. Although many people may have risk factors for the disease, very few actually develop it.

• #46 EGPA (Formerly Churg-Strauss Syndrome): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/churg-strauss-syndrome-eosinophilic-granulomatosis-with-polyangiitis-egpa

  Researchers have grouped EGPA together with a handful of similar conditions that all involve a certain autoimmune antibody, called ANCA. They call these conditions ANCA-associated vasculitis, or AAV. However, only about 40% of people with EGPA have the ANCA antibody, so its not the only factor. […] Eosinophilia seems to be a separate cause of symptoms that often occur long before vasculitis does. When eosinophils accumulate in your tissues, they trigger inflammation and damage them. Eosinophilia is related to asthma and allergies. People with asthma are 34 times more at risk of EGPA than others.

• #47 Vasculitis

  https://www.nhs.uk/conditions/vasculitis/

  Eosinophilic granulomatosis with polyangiitis, also called Churg-Strauss syndrome, is a type of vasculitis that mainly affects adults around 38 to 54. […] It can also affect the nerves, causing weakness, pins and needles or numbness, and sometimes damages the kidneys or heart muscle. […] Eosinophilic granulomatosis with polyangiitis is usually treated with steroid medicine.

• #48 Churg-Strauss Syndrome: Causes, Symptoms, Diagnosis & Treatment

  https://www.medindia.net/health/conditions/churg-strauss-syndrome.htm

  Churg-Strauss syndrome is a condition characterized by blood vessel inflammation that can restrict blood flow to vital organs and tissues. In some cases it can lead to permanent damage of these organs. […] Churg-Strauss syndrome is usually diagnosed in people between ages 38 and 52. It is almost absent in children and older people. Another risk factor for Churg-Strauss syndrome is having a history of severe nasal allergies, chronic sinusitis or asthma. […] Churg-Strauss syndrome is a rare disease and the number of people with the syndrome has not been clearly estimated. Although many people may have risk factors for the disease, very few actually develop it.

• #49 Eosinophilic Granulomatosis with Polyangiitis (EGPA) – Brigham and Women’s Hospital

  https://www.brighamandwomens.org/lung-center/diseases-and-conditions/eosinophilic-granulomatosis-with-polyangiitis

  A rare systemic vasculitis affecting less than two out of every million people each year, eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a condition that causes inflammation of small-sized blood vessels in people with asthma. […] The cause of EGPA is unknown. Potential risk factors may include: […] Asthma, allergic rhinitis and/or nasal polyps […] Drug sensitivities to penicillin, penicillamine, iodides, leukotriene modifiers or mesalazine […] Environmental exposure to inhaled allergens such as silica dust.

• #50 Eosinophilic granulomatosis with polyangiitis. Churg-Strauss syndrome

  https://dermnetnz.org/topics/eosinophilic-granulomatosis-with-polyangiitis

  Eosinophilic granulomatosis with polyangiitis is a rare disease that affects the lungs, the skin and sometimes other organs. […] The cause of eosinophilic granulomatosis with polyangiitis is uncertain, but it is currently thought to be an autoimmune process. […] It has been associated with drug sensitivities to penicillin, penicillamine, iodides, leukotriene modifiers and mesalazine in some patients.

• #51 Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis | Nature Reviews Rheumatology

  https://www.nature.com/articles/s41584-023-00958-w

  Eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome) is a rare small-vessel vasculitis that occurs in patients with asthma and eosinophilia and is histologically characterized by tissue eosinophilia, necrotizing vasculitis and eosinophil-rich granulomatous inflammation. […] The pathogenesis of EGPA is driven by genetic and environmental factors. Genetic studies have highlighted associations between HLA-DQ and MPO-ANCA-positive EGPA, whereas ANCA-negative EGPA is mainly associated with genetic variants involved in mucosal responses and eosinophil biology, such as GPA33 and IL5. Several other variants linked to asthma and eosinophil counts in the general population are associated with the whole EGPA spectrum. […] Among environmental factors, exposure to silica, organic solvents and farming was associated with an increased risk of EGPA, whereas cigarette smoking was associated with a lower risk. How genetics and environment interact to shape the susceptibility to and the phenotype of EGPA is still unclear.

• #52 Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis | Nature Reviews Rheumatology

  https://www.nature.com/articles/s41584-023-00958-w

  Eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome) is a rare small-vessel vasculitis that occurs in patients with asthma and eosinophilia and is histologically characterized by tissue eosinophilia, necrotizing vasculitis and eosinophil-rich granulomatous inflammation. […] The pathogenesis of EGPA is driven by genetic and environmental factors. Genetic studies have highlighted associations between HLA-DQ and MPO-ANCA-positive EGPA, whereas ANCA-negative EGPA is mainly associated with genetic variants involved in mucosal responses and eosinophil biology, such as GPA33 and IL5. Several other variants linked to asthma and eosinophil counts in the general population are associated with the whole EGPA spectrum. […] Among environmental factors, exposure to silica, organic solvents and farming was associated with an increased risk of EGPA, whereas cigarette smoking was associated with a lower risk. How genetics and environment interact to shape the susceptibility to and the phenotype of EGPA is still unclear.

• #53 Eosinophilic Granulomatosis with Polyangiitis – Apfed

  https://apfed.org/about-ead/eosinophilic-granulomatosis-with-polyangiitis/

  Eosinophilic Granulomatosis with Polyangiitis (EGPA), formerly known as Churg-Strauss Syndrome, is a rare autoimmune disorder characterized by inflammation of blood vessels and the presence of high numbers of white blood cells known as eosinophils. […] The cause of EGPA is not known, although it is believed to result from an interaction of genetics and the environment, complicated by an overactive immune system. […] Because all patients with EGPA have high levels of eosinophils at some point during their disease, it is thought that there may be some dysregulation of eosinophil production, maturation, or development. […] Anti-neutrophil cytoplasmic antibodies (ANCA) are also present in many patients with EGPA and may also play a role in EGPA disease development. […] While the exact role of ANCA in EGPA and other vasculitides remains unclear, it is felt that the binding of ANCA to vascular walls contributes to vascular inflammation and injury as well as chemotaxis of inflammatory cells.

• #54 Cardiac Involvement in Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) | Society for Cardiovascular Magnetic Resonance

  https://scmr.org/cases-of-scmr/number-24-04/

  Churg-Strauss vasculitis (also called Eosinophilic granulomatosis with polyangiitis) is characterized by necrotizing vasculitis of small and medium-sized systemic blood vessels described in 1951 by Drs. Jacob Churg and Lotte Strauss. […] Cardiac involvement is caused by both mediators released from activated eosinophils as well as from coronary arteries vasculitis. Myocarditis (in acute phase), leads to post-inflammatory fibrosis and restrictive cardiomyopathy, followed by congestive cardiac failure. Cardiac involvement is associated with a poor prognosis, and therefore early diagnosis and institution of treatment is imperative. […] Two phenotypes of the disease have been recognized – those with ANCA positive have a vasculitis phenotype and present with myalgia, migrating polyarthralgia, weight loss, mononeuritis multiplex, and renal involvement; however, those who are ANCA negative have an eosinophilic phenotype with a higher incidence of myocarditis. […] However, cardiac involvement is present in more than 50% of patients with EGPA, which is the major cause of morbidity and mortality.

• #55 Eosinophilic Granulomatosis with Polyangiitis formally Churg Strauss Syndrome — Vasculitis Ireland Awareness (VIA)

  https://www.vasculitis-ia.org/vasculitis-types/eosinophilic-granulomatosis-with-polyangiitis-formally-churg-strauss-syndrome

  Eosinophilic Granulomatosis with Polyangiitis (EGPA), previously known as Churg-Strauss Syndrome, is an inflammatory disease of small and medium sized blood vessels. […] The cause of EGPA is not definitively known, and it is likely multifactorial, involving a combination of genetic, environmental, and immunological factors. Genetic studies have identified certain genes associated with EGPA, and the presence of Anti Neutrophil Cytoplasmic Antibodies (ANCA) has been observed in some individuals, suggesting a potential role of autoimmune mechanisms in the disease process. Furthermore, abnormalities in certain cytokines, which are hormone-like chemicals involved in immune responses, have also been implicated in EGPA. […] However, further research is needed to fully understand the complex interplay of these factors in the development and progression of EGPA.

• #56 Learn About Eosinophilic Granulomatosis with Polyangiitis (EGPA) | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/egpa/about-epga

  EGPA is an ultra-rare disease, affecting an estimated 5,000 individuals in the United States. […] It is not known what causes EGPA. Typically, the immune system defends the body but in auto-immune diseases, such as vasculitis, the immune system increases activity and attacks healthy tissues for reasons we dont quite understand.

• #57 Eosinophilic Granulomatosis with Polyangiitis – Vasculitis Foundation

  https://vasculitisfoundation.org/education/vasculitis-types/eosinophilic-granulomatosis-with-polyangiitis/

  Eosinophilic granulomatosis with polyangiitis (EGPA), formerly called Churg-Strauss syndrome, is a form of vasculitis […] The cause of EGPA is not fully understood. Researchers believe an infection may set the inflammatory process in motion. Environmental factors such as inhaled allergens, or certain medications or vaccinations, may also play a role in starting EGPA or in its return. Family history and genetics may also cause it.

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