Materiały źródłowe

• #1 Churg-Strauss syndrome – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/churg-strauss-syndrome/diagnosis-treatment/drc-20353765

  To diagnose Churg-Strauss syndrome, doctors usually request several types of tests, including: […] A blood test can detect certain antibodies in your blood that can suggest, but not confirm, a diagnosis of Churg-Strauss syndrome. […] X-rays and CT scans can reveal abnormalities in your lungs and sinuses. […] If other tests suggest Churg-Strauss syndrome, you might have a small sample of tissue removed for examination under a microscope. […] If you have signs and symptoms common to Churg-Strauss syndrome, make an appointment with your doctor. Early diagnosis and treatment significantly improve the outlook of this condition. […] You may be referred to a doctor who specializes in disorders that cause blood vessel inflammation (vasculitis), such as a rheumatologist or immunologist. […] Basic questions to ask your doctor might include: What diagnostic tests do I need? […] A doctor who sees you for possible Churg-Strauss syndrome is likely to ask you questions, such as: Have your symptoms, especially asthma-related, worsened over time?

• #2 How You Get a Diagnosis of Churg-Strauss Syndrome

  https://www.webmd.com/lung/churg-strauss-diagnosis

  It can take a few steps to find out whether you have Churg-Strauss syndrome. This rare disease is hard to diagnose based on its typical symptoms like fever, asthma, appetite loss, and tiredness. The trouble is that many conditions can cause these same symptoms. […] There also isn’t a single test to diagnose Churg-Strauss syndrome. Your doctor will look at many different things, including your symptoms, a physical exam, and the results of your lab and imaging tests. […] The American College of Rheumatology has set up six „criteria” — or standards — for diagnosing Churg-Strauss syndrome. These are signs that most people with the condition have. […] Your doctor will diagnose you with Churg-Strauss syndrome if you meet four of these six criteria: […] Doctors diagnose Churg-Strauss syndrome using one or more of these tests:

• #3 Churg-Strauss Disease Workup: Approach Considerations, Laboratory Studies, Imaging Studies

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

  Where possible, the diagnosis is confirmed by demonstration of angiographic abnormalities in affected organs and by pathognomonic biopsy findings. […] According to the American College of Rheumatology criteria, clinical diagnosis is established when 4 of the following manifestations are documented: (1) allergic history, (2) asthma, (3) eosinophilia, (4) migratory pulmonary infiltrates, (5) paranasal sinus abnormality, (6) mononeuropathy or polyneuropathy, and (7) demonstration of extravascular eosinophilic infiltration of tissues on biopsy. […] Once the second phase of EGPA is reached, eosinophilia ( 1500/L or 10% of total peripheral WBCs) is found on the peripheral blood film of at least 90% of untreated patients. […] Treatment of asthmatic manifestations of the first stage of EGPA with corticosteroids may promptly resolve this characteristic finding. The prompt resolution of eosinophilia with corticosteroid treatment is itself characteristic of EGPA.

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

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

  Eosinophilic granulomatosis with polyangiitis (EGPA) or, as it was formerly known, Churg-Strauss syndrome, is one of the antineutrophil cytoplasmic antibody (ANCA)associated vasculitides. It is a rare systemic necrotizing granulomatous vasculitis that affects small- to medium-sized vessels and can result in life-threatening multiorgan damage. […] In 1990, the American College of Rheumatology (ACR) proposed the following criteria for the classification of EGPA: Asthma (wheezing, expiratory rhonchi), Eosinophilia of more than 10% on a differential white blood cell count (WBC), Paranasal sinusitis, Pulmonary infiltrates (may be transient), Histologic proof of vasculitis with extravascular eosinophils, Mononeuritis multiplex or polyneuropathy. […] The presence of four or more criteria yields a sensitivity of 85% and a specificity of 99.7%.

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

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

  How is EGPA diagnosed? […] It is the specific combination of symptoms and signs, the pattern of organ involvement, and the presence of certain abnormal blood tests (eosinophilia, in particular) that help the doctor make the diagnosis. […] In addition to a detailed history and physical examination, blood tests, chest X-rays and other types of imaging studies, nerve conduction tests, and tissue biopsies (e.g., of lung, skin, or nerve) may be performed to help diagnose EGPA. […] The following features are consistent with a diagnosis of GPA: asthma, eosinophilia [10% on differential WBC count], mononeuropathy, transient pulmonary infiltrates on chest X-rays, paranasal sinus abnormalities, biopsy containing a blood vessel with extravascular eosinophils.

• #6 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. […] Steps for diagnosing Churg-Strauss syndrome include: clinical evaluation, characteristic physical findings, specialized tests. […] Specialized tests may involve biopsy and microscopic examination of lung tissue samples. […] To receive a diagnosis of Churg-Strauss syndrome, a person must meet at least four of the following six criteria: asthma, eosinophilia levels of at least 10% within circulating blood, mono- or polyneuropathy (when peripheral nerves are damaged), nonfixed pulmonary infiltrates (when substances such as blood or pus are present in the lungs), paranasal sinus abnormalities, such as soft tissue masses like polyps, extravascular eosinophilia (a high number of white blood cells outside the vascular system).

• #7 Churg-Strauss Syndrome with Orbital Inflammation

  https://webeye.ophth.uiowa.edu/eyeforum/cases/119-churg-strauss-syndrome.htm

  The American College of Rheumatology set forth the most widely utilized criteria for diagnosing and classifying CSS; the presence of 4 or more criteria yields a sensitivity of 85% and a specificity of 99.7% (Table 1) (Masi, 1990). […] The presence of 4 or more criteria yields a sensitivity of 85% and a specificity of 99.7%.

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

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

  Eosinophilic granulomatosis with polyangiitis (EGPA) or, as it was formerly known, Churg-Strauss syndrome, is one of the antineutrophil cytoplasmic antibody (ANCA)associated vasculitides. It is a rare systemic necrotizing granulomatous vasculitis that affects small- to medium-sized vessels and can result in life-threatening multiorgan damage. […] In 1990, the American College of Rheumatology (ACR) proposed the following criteria for the classification of EGPA: Asthma (wheezing, expiratory rhonchi), Eosinophilia of more than 10% on a differential white blood cell count (WBC), Paranasal sinusitis, Pulmonary infiltrates (may be transient), Histologic proof of vasculitis with extravascular eosinophils, Mononeuritis multiplex or polyneuropathy. […] The presence of four or more criteria yields a sensitivity of 85% and a specificity of 99.7%.

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

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

  Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, belongs to a group of diseases characterized by necrotizing vasculitis affecting small- and medium-sized systemic blood vessels, also known as antineutrophil cytoplasmic autoantibody (ANCA)associated vasculitis (AAV). […] Although EGPA is classified as an ANCA-associated vasculitis, ANCA is positive in only 30% to 40% of cases, making diagnosis challenging. […] Clinical recognition of multisystem involvement, especially in patients with eosinophilic asthma, is key to diagnosis. […] The management of EGPA also differs from other AAVs, requiring tailored treatment strategies. […] The scoring system developed by the ACR and European Alliance of Associations for Rheumatology (ACR-EULAR) in 2022 for diagnosing EGPA includes: Maximum eosinophil count of 1109 cells/L or higher (+5), Obstructive airway disease (+3), Nasal polyps (+3), ANCA or anti-proteinase 3-ANCA positivity (3), Extravascular eosinophilic predominant inflammation (+2), Mononeuritis multiplex or motor neuropathy not due to radiculopathy (+1), Hematuria (1).

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

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

  In 2022 the ACR and the European Alliance of Associations for Rheumatology (EULAR) updated the classification with a score that uses weighted criteria and also includes ANCA serology testing. A score of 6 or higher (positive minus negative points) has a sensitivity of 85% and a specificity of 99% for diagnosis of EGPA. […] It is essential to recognize that the negative points for hematuria (-1) and c-ANCA (-3), in the 2022 ACR-EULAR criteria do not mean that their presence excludes EGPA. Rather, it serves to signify their infrequency in EGPA. If either criterion is present, the clinician should definitively rule out other small-vessel vasculitides, in particular granulomatosis with polyangiitis (GPA), before settling on EGPA as the diagnosis. […] Note that the ACR and ACR-EULAR criteria were developed for the purpose of classification; they were not intended for diagnosis and should not be used as diagnostic criteria. A proposed but unvalidated approach, used in the pivotal Mepolizumab in Relapsing or Refractory EGPA (MIRRA) trial, bases the diagnosis of EGPA on the presence of asthma and eosinophilia (1000 cells/mm3 or 10% of WBC), along with at least two of the following criteria: Biopsy showing histopathologic evidence of eosinophilic vasculitis, perivascular eosinophilic infiltration, or eosinophilrich granulomatous inflammation, Neuropathy, mono or poly (motor deficit or nerve conduction abnormality), Pulmonary infiltrates, nonfixed, Sinonasal abnormality, Cardiomyopathy (established by echocardiography or MRI), Glomerulonephritis (hematuria, red cell casts, proteinuria), Alveolar hemorrhage (by bronchoalveolar lavage), Palpable purpura, Positive test for ANCA (MPO or PR3). […] See Presentation and Workup.

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

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

  Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is a specific variant within a group of diseases characterized by necrotizing vasculitis of small- and medium-sized systemic blood vessels. […] In 1990, the American College of Rheumatology (ACR) proposed new classification criteria for EGPA, requiring the presence of 4 out of 6 features, as mentioned below, for diagnosis. […] At the Chapel Hill Consensus Conference in 1994, EGPA was defined as „eosinophil-rich and granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small- to medium-sized vessels, associated with asthma and eosinophilia.” […] The scoring system developed by the ACR and European Alliance of Associations for Rheumatology (ACR-EULAR) in 2022 for diagnosing EGPA includes: Maximum eosinophil count of 1109 cells/L or higher (+5), Obstructive airway disease (+3), Nasal polyps (+3), ANCA or anti-proteinase 3-ANCA positivity (3), Extravascular eosinophilic predominant inflammation (+2), Mononeuritis multiplex or motor neuropathy not due to radiculopathy (+1), Hematuria (1).

• #12 Identifying EGPA | NUCALA (mepolizumab) for HCPs

  https://nucalahcp.com/egpa/egpa-patient-types/identifying-egpa/

  Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare disease defined by eosinophil-rich and necrotizing granulomatous inflammation, and necrotizing vasculitis predominately affecting small to medium vessels. […] In these classification criteria, for patients with vasculitis: a cumulative score of 6 points yields an 85% sensitivity and 99% specificity for EGPA. […] The 2022 ACR/EULAR classification criteria are meant for classification and not diagnostic purposes. These criteria should be applied when a diagnosis of small- or medium-vessel vasculitis has been made. Alternative diagnoses mimicking vasculitis should be excluded prior to applying these criteria. […] Although biopsy is the gold standard, most patients do not undergo biopsy and are diagnosed on a clinical basis.

• #13 Eosinophilic Granulomatosis with Polyangiitis (EGPA) – Musculoskeletal and Connective Tissue Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/vasculitis/eosinophilic-granulomatosis-with-polyangiitis-egpa

  Eosinophilic granulomatosis with polyangiitis is a systemic small- and medium-vessel necrotizing vasculitis, characterized by extravascular granulomas, eosinophilia, and tissue infiltration by eosinophils. Diagnosis is best confirmed by biopsy. […] Diagnosis of EGPA is suggested by clinical findings and results of routine laboratory tests but should usually be confirmed by biopsy of lung or other affected tissue. […] The 2012 Chapel Hill Consensus Conference defined eosinophilic granulomatosis with polyangiitis (EGPA) as an eosinophil-rich and necrotizing granulomatous inflammation involving the respiratory tract with necrotizing vasculitis of small- and medium-sized vessels in association with asthma and eosinophilia. […] A score of 6 has a sensitivity of 85% and a specificity of 99%.

• #14 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

  Statement 3: The diagnostic evaluation of patients with suspected EGPA should always be multidisciplinary; it should rule out other eosinophilic and vasculitic disorders and investigate the main disease complications, particularly heart, respiratory, skin, renal and nervous system involvement, along with ANCA and eosinophilia. Biopsy is recommended when feasible, but is not essential to make the diagnosis of EGPA. (L: 3b; G: C) […] Statement 4: ANCA testing should be performed in all patients with suspected EGPA. ANCA are detectable in 30-40% of patients with EGPA, most of whom test positive for MPO-ANCA. MPO-ANCA-positive patients frequently show vasculitis features, such as glomerulonephritis, neuropathy and purpura, whereas ANCA-negative patients more frequently manifest cardiomyopathy and lung involvement. (L: 2a; G: B)

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

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

  In 2022 the ACR and the European Alliance of Associations for Rheumatology (EULAR) updated the classification with a score that uses weighted criteria and also includes ANCA serology testing. A score of 6 or higher (positive minus negative points) has a sensitivity of 85% and a specificity of 99% for diagnosis of EGPA. […] It is essential to recognize that the negative points for hematuria (-1) and c-ANCA (-3), in the 2022 ACR-EULAR criteria do not mean that their presence excludes EGPA. Rather, it serves to signify their infrequency in EGPA. If either criterion is present, the clinician should definitively rule out other small-vessel vasculitides, in particular granulomatosis with polyangiitis (GPA), before settling on EGPA as the diagnosis. […] Note that the ACR and ACR-EULAR criteria were developed for the purpose of classification; they were not intended for diagnosis and should not be used as diagnostic criteria. A proposed but unvalidated approach, used in the pivotal Mepolizumab in Relapsing or Refractory EGPA (MIRRA) trial, bases the diagnosis of EGPA on the presence of asthma and eosinophilia (1000 cells/mm3 or 10% of WBC), along with at least two of the following criteria: Biopsy showing histopathologic evidence of eosinophilic vasculitis, perivascular eosinophilic infiltration, or eosinophilrich granulomatous inflammation, Neuropathy, mono or poly (motor deficit or nerve conduction abnormality), Pulmonary infiltrates, nonfixed, Sinonasal abnormality, Cardiomyopathy (established by echocardiography or MRI), Glomerulonephritis (hematuria, red cell casts, proteinuria), Alveolar hemorrhage (by bronchoalveolar lavage), Palpable purpura, Positive test for ANCA (MPO or PR3). […] See Presentation and Workup.

• #16 How You Get a Diagnosis of Churg-Strauss Syndrome

  https://www.webmd.com/lung/churg-strauss-diagnosis

  Your doctor may order them to measure the number of eosinophils in your blood, or to check for signs of inflammation in your body. High eosinophil levels can mean that you have Churg-Strauss syndrome or asthma. […] A blood test called ANCA looks for these autoantibodies. Only about 30% to 50% of people with Churg-Strauss syndrome will have a positive ANCA test, so it alone can’t confirm the diagnosis. […] If other tests suggest that you might have Churg-Strauss syndrome, your doctor will confirm the results with a biopsy. The doctor removes a small piece of tissue from your lungs, skin, or other organs. Then a lab checks the sample under a microscope for signs of inflammation or damage. […] Biopsy results in people with Churg-Strauss syndrome often show: High numbers of eosinophils, Clusters of immune cells called a granuloma, Blood vessel damage caused by immune cells, called vasculitis.

• #17 Churg-Strauss Disease Workup: Approach Considerations, Laboratory Studies, Imaging Studies

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

  Where possible, the diagnosis is confirmed by demonstration of angiographic abnormalities in affected organs and by pathognomonic biopsy findings. […] According to the American College of Rheumatology criteria, clinical diagnosis is established when 4 of the following manifestations are documented: (1) allergic history, (2) asthma, (3) eosinophilia, (4) migratory pulmonary infiltrates, (5) paranasal sinus abnormality, (6) mononeuropathy or polyneuropathy, and (7) demonstration of extravascular eosinophilic infiltration of tissues on biopsy. […] Once the second phase of EGPA is reached, eosinophilia ( 1500/L or 10% of total peripheral WBCs) is found on the peripheral blood film of at least 90% of untreated patients. […] Treatment of asthmatic manifestations of the first stage of EGPA with corticosteroids may promptly resolve this characteristic finding. The prompt resolution of eosinophilia with corticosteroid treatment is itself characteristic of EGPA.

• #18 Symptoms and Diagnosis of Eosinophilic Granulomatosis with Polyangiitis (EGPA) | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/egpa/symptoms-diagnosis

  EGPA can affect blood vessels in many different organs so there are a wide range of symptoms depending on which organs are affected. […] Diagnosing EGPA will require a series of steps starting with a detailed history to uncover the unique combination of symptoms and signs that may lead your doctor to suspect EGPA and diagnostic tests that will confirm the prognosis. A typical path to diagnosis will look similar to these steps: A clinical exam, where your doctor will ask about your medical history and do a physical exam. Your doctor will look for a history of asthma, allergies and other EGPA signs and symptoms. […] Blood tests to check your white blood count and determine if eosinophils percentages are greater than 5% of your total white blood cells. […] An urinalysis to determine if there are too many red blood cells or protein in your urine. […] A chest X-ray or CT scan to look for abnormalities in the lungs or sinuses. […] A biopsy, also called a tissue sample, may be ordered if abnormal findings are seen in the above tests to look for eosinophils, eosinophilic granulomas and/or vasculitis.

• #19 How You Get a Diagnosis of Churg-Strauss Syndrome

  https://www.webmd.com/lung/churg-strauss-diagnosis

  Your doctor may order them to measure the number of eosinophils in your blood, or to check for signs of inflammation in your body. High eosinophil levels can mean that you have Churg-Strauss syndrome or asthma. […] A blood test called ANCA looks for these autoantibodies. Only about 30% to 50% of people with Churg-Strauss syndrome will have a positive ANCA test, so it alone can’t confirm the diagnosis. […] If other tests suggest that you might have Churg-Strauss syndrome, your doctor will confirm the results with a biopsy. The doctor removes a small piece of tissue from your lungs, skin, or other organs. Then a lab checks the sample under a microscope for signs of inflammation or damage. […] Biopsy results in people with Churg-Strauss syndrome often show: High numbers of eosinophils, Clusters of immune cells called a granuloma, Blood vessel damage caused by immune cells, called vasculitis.

• #20 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

  Statement 7: For remission induction in patients with new-onset, active EGPA, glucocorticoids should be administered as initial therapy. In patients with severe disease (unfavorable prognostic factors discussed in Statement 6) cyclophosphamide or, as an alternative, rituximab, should be added. In patients with non-severe disease, glucocorticoids alone should be used. (L: 2b; G: B) […] Statement 8: For remission maintenance, in patients with severe EGPA, we recommend using rituximab, mepolizumab or traditional DMARDs in combination with glucocorticoids. In patients with non-severe EGPA, we suggest glucocorticoids, alone or in combination with mepolizumab. Glucocorticoids should be tapered to the minimum effective dosage to reduce toxicity. (L: 2b; G: B) […] Statement 9: EGPA relapse is defined as the recurrence of clinical signs or symptoms attributable to active disease following a period of remission. The need for an increase in the glucocorticoid dosage or the initiation of or an increase in an immunosuppressant should also be considered a relapse. The relapse or new onset of systemic vasculitis (systemic relapse) should be differentiated from the isolated exacerbation of asthma and ENT manifestations (respiratory relapse). (L: 5; G: D)

• #21 Churg-Strauss Disease Workup: Approach Considerations, Laboratory Studies, Imaging Studies

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

  Diagnosis of Churg-Strauss disease (CSD), now known as eosinophilic granulomatosis with polyangiitis (EGPA), is in many cases a process comprised of several tests, clinical visits, and high suspicion. The main reason for this relatively cumbersome diagnosis process is that many of the symptoms and signs change over time, hence delaying clear diagnosis. There is no gold standard diagnostic tool. For all these reasons, EGPA diagnosis is mainly clinical with support of lab work and sometimes histopathology and biopsy. As such, when a patient presents with eosinophilic asthma, the clinician must look for a pattern of multisystem disease and investigate for other supportive findings. […] Peripheral blood eosinophilia (greater than 10% on differential white blood cell count or greater than 1500 /dl) is the best-known lab hallmark of the disease. Elevated serum IgE is also found in 75% of patients.

• #22 Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-us/942

  Eosinophilic granulomatosis with polyangiitis (also known as Churg-Strauss syndrome) is characterised by the pathological triad of tissue eosinophilia, granulomatous inflammation, and vasculitis. […] Typically patients are initially diagnosed with asthma, and then months to years later develop manifestations due to tissue eosinophilia, followed by vasculitis. […] In EGPA, vasculitis is associated with asthma and eosinophilia. […] Affected people may have perinuclear anti-neutrophil cytoplasmic antibodies directed against myeloperoxidase. […] Key diagnostic factors include history of asthma, allergic rhinitis, or sinusitis, focal numbness or weakness, nasal discharge or stuffiness, or facial pain, palpable purpura and petechiae, wheeze, haemoptysis, and skin nodules. […] 1st investigations to order include FBC with differential, serum anti-neutrophil cytoplasmic antibodies (ANCA), serum CRP, erythrocyte sedimentation rate, serum urea and creatinine, urinalysis, pulmonary function test, chest x-ray, and echocardiogram.

• #23 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

  How Do Medical Professionals Diagnose Churg-Strauss Syndrome? A patient must meet at least four out of six criteria to diagnose Churg-Strauss syndrome (eosinophilic granulomatosis with polyangiitis syndrome): Asthma, Abnormal blood tests, including a type of high white blood cells (eosinophilia), Sinus infection (sinusitis), Pneumonia, Biopsy that shows vasculitis with eosinophils, Evidence of nerve dysfunction. […] Tests that may be indicated to help diagnose Churg-Strauss syndrome include the following: Complete blood count (CBC), Peripheral blood smear, Blood tests for certain infections, including HIV, Aspergillus, and toxocariasis; tryptase; vitamin B12 levels; erythrocyte sedimentation rate (ESR); C-reactive protein (CRP) levels; blood urea nitrogen (BUN) and serum creatinine levels; IgE levels; eosinophil cationic protein (ECP); soluble interleukin-2 receptor (sIL-2R); and soluble thrombomodulin (sTM).

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

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

  In 2022 the ACR and the European Alliance of Associations for Rheumatology (EULAR) updated the classification with a score that uses weighted criteria and also includes ANCA serology testing. A score of 6 or higher (positive minus negative points) has a sensitivity of 85% and a specificity of 99% for diagnosis of EGPA. […] It is essential to recognize that the negative points for hematuria (-1) and c-ANCA (-3), in the 2022 ACR-EULAR criteria do not mean that their presence excludes EGPA. Rather, it serves to signify their infrequency in EGPA. If either criterion is present, the clinician should definitively rule out other small-vessel vasculitides, in particular granulomatosis with polyangiitis (GPA), before settling on EGPA as the diagnosis. […] Note that the ACR and ACR-EULAR criteria were developed for the purpose of classification; they were not intended for diagnosis and should not be used as diagnostic criteria. A proposed but unvalidated approach, used in the pivotal Mepolizumab in Relapsing or Refractory EGPA (MIRRA) trial, bases the diagnosis of EGPA on the presence of asthma and eosinophilia (1000 cells/mm3 or 10% of WBC), along with at least two of the following criteria: Biopsy showing histopathologic evidence of eosinophilic vasculitis, perivascular eosinophilic infiltration, or eosinophilrich granulomatous inflammation, Neuropathy, mono or poly (motor deficit or nerve conduction abnormality), Pulmonary infiltrates, nonfixed, Sinonasal abnormality, Cardiomyopathy (established by echocardiography or MRI), Glomerulonephritis (hematuria, red cell casts, proteinuria), Alveolar hemorrhage (by bronchoalveolar lavage), Palpable purpura, Positive test for ANCA (MPO or PR3). […] See Presentation and Workup.

• #25 Symptoms and Diagnosis of Eosinophilic Granulomatosis with Polyangiitis (EGPA) | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/egpa/symptoms-diagnosis

  EGPA can affect blood vessels in many different organs so there are a wide range of symptoms depending on which organs are affected. […] Diagnosing EGPA will require a series of steps starting with a detailed history to uncover the unique combination of symptoms and signs that may lead your doctor to suspect EGPA and diagnostic tests that will confirm the prognosis. A typical path to diagnosis will look similar to these steps: A clinical exam, where your doctor will ask about your medical history and do a physical exam. Your doctor will look for a history of asthma, allergies and other EGPA signs and symptoms. […] Blood tests to check your white blood count and determine if eosinophils percentages are greater than 5% of your total white blood cells. […] An urinalysis to determine if there are too many red blood cells or protein in your urine. […] A chest X-ray or CT scan to look for abnormalities in the lungs or sinuses. […] A biopsy, also called a tissue sample, may be ordered if abnormal findings are seen in the above tests to look for eosinophils, eosinophilic granulomas and/or vasculitis.

• #26 Churg-Strauss syndrome – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/churg-strauss-syndrome/diagnosis-treatment/drc-20353765

  To diagnose Churg-Strauss syndrome, doctors usually request several types of tests, including: […] A blood test can detect certain antibodies in your blood that can suggest, but not confirm, a diagnosis of Churg-Strauss syndrome. […] X-rays and CT scans can reveal abnormalities in your lungs and sinuses. […] If other tests suggest Churg-Strauss syndrome, you might have a small sample of tissue removed for examination under a microscope. […] If you have signs and symptoms common to Churg-Strauss syndrome, make an appointment with your doctor. Early diagnosis and treatment significantly improve the outlook of this condition. […] You may be referred to a doctor who specializes in disorders that cause blood vessel inflammation (vasculitis), such as a rheumatologist or immunologist. […] Basic questions to ask your doctor might include: What diagnostic tests do I need? […] A doctor who sees you for possible Churg-Strauss syndrome is likely to ask you questions, such as: Have your symptoms, especially asthma-related, worsened over time?

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

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

  How is EGPA Diagnosed? Your doctor will discuss your symptoms. There are six specific ones (they’ll call them criteria) they’re looking for to help make a diagnosis. They might suspect you have EGPA if you only have some of these symptoms, but it takes four out of the six to feel confident of the diagnosis: […] There’s no single test to diagnose EGPA. Your doctor will look at your medical history and give you a physical exam. They’ll ask you about your symptoms, especially asthma. […] You may also get: […] Blood tests: The doctor will look for proteins in your blood that show inflammation. They’ll also check for extra white blood cells. […] Imaging tests: These could include an X-ray, CT scan, and MRI to check for problems in your lungs and sinuses. […] Biopsy: The doctor will take a small tissue sample from an affected spot, like an area of your skin with a rash, to look for inflamed blood vessels. Or they might do a kidney or lung biopsy.

• #28 How You Get a Diagnosis of Churg-Strauss Syndrome

  https://www.webmd.com/lung/churg-strauss-diagnosis

  It can take a few steps to find out whether you have Churg-Strauss syndrome. This rare disease is hard to diagnose based on its typical symptoms like fever, asthma, appetite loss, and tiredness. The trouble is that many conditions can cause these same symptoms. […] There also isn’t a single test to diagnose Churg-Strauss syndrome. Your doctor will look at many different things, including your symptoms, a physical exam, and the results of your lab and imaging tests. […] The American College of Rheumatology has set up six „criteria” — or standards — for diagnosing Churg-Strauss syndrome. These are signs that most people with the condition have. […] Your doctor will diagnose you with Churg-Strauss syndrome if you meet four of these six criteria: […] Doctors diagnose Churg-Strauss syndrome using one or more of these tests:

• #29 What Is Churg-Strauss Syndrome?

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

  Pulmonary Function Test (PFT) – PFT shows lung (airflow) obstruction. […] CT (Computed Tomography) of the Chest and Sinus – CT of the chest and sinus shows thickening of the nasal membrane and sinus walls, lung infiltrates, bronchial wall thickening, […] Sural Nerve Biopsy – Axonal degeneration is present indicative of peripheral neuropathy. Sural nerve biopsy is regarded as the gold standard for Churg-Strauss diagnosis.

• #30 Eosinophilic Granulomatosis Polyangiitis (EGPA) – EyeWiki

  https://eyewiki.org/Eosinophilic_Granulomatosis_Polyangiitis_(EGPA)

  Migratory infiltrates on chest radiographs are considered one of the key features of EGPA. High-resolution computed tomography (CT) scans are more sensitive to lung parenchymal changes. […] The ACR diagnostic criteria for EGPA include maximum eosinophil count 1×109 / liter (+5), obstructive airway disease (+3), nasal polyps (+3), cytoplasmic antineutrophil cytoplasmic antibody (ANCA) or antiproteinase 3 ANCA positivity (-3), extravascular eosinophilic predominant inflammation (+2), mononeuritis multiplex/motor neuropathy not due to radiculopathy (+1), and hematuria (-1). […] Treatment with immunosuppression followed by maintenance therapy may be required for EGPA.

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

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

  Given the history of bronchial asthma, ACS- NSTEMI and neuropathy (proximal muscle weakness & pain), hypereosinophillia, increased IgE, increased CRP, increased ESR and positive rheumatoid factor, a diagnosis of Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) was made. […] CMR is the considered the gold standard noninvasive diagnostic test for early diagnosis and rapid institution of therapy. […] This case highlights the importance of CMR in early diagnosis of cardiomyopathy, and subclassify the exact etiology of non-ischemic pattern of cardiac involvement, albeit in appropriate clinical context.

• #32 Computed Tomography Angiogram in Diagnosis of Churg-Strauss Vasculitis | The Journal of Rheumatology

  https://www.jrheum.org/content/36/12/2851

  A novel diagnostic approach may be a helpful adjunct to clinical judgment in evaluating Churg-Strauss syndrome. […] The diagnosis of Churg-Strauss is based on a biopsy of affected tissues, traditionally an open-lung biopsy. […] Following the ACR diagnostic criteria, presence of 4 of the following 6 criteria makes the diagnosis with 99.7% specificity: (1) asthma; (2) eosinophilia 10%; (3) mononeuropathy or polyneuropathy; (4) migratory or transient pulmonary opacities; (5) paranasal sinus abnormality; (6) biopsy showing accumulation of eosinophils in extravascular areas. […] While highly specific, these diagnostic criteria suffer from poor sensitivity since many of the symptoms are fleeting. […] An evaluation of the literature reveals a paucity of data regarding the use of CTA in the Churg-Strauss syndrome. […] In addition to the prototypical presentation of a rare vasculitis, our case demonstrates a novel diagnostic approach that may be a helpful adjunct to clinical judgment in evaluating Churg-Strauss syndrome.

• #33 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 most up to date diagnostic rules, written by the European Respiratory Society (ERS), say that someone with EGPA should have: […] Blood tests will show high levels of Eosinophil white blood cells in patients with active EGPA. […] People with active EGPA may have patches of vasculitic damage where eosinophils invade and damage the lung tissue (infiltrates) that may be visible on a chest x-ray. […] All patients diagnosed with EGPA have asthma, and this will almost always be shown through spirometry (breathing tests) that show an inability to breathe out a quickly as should be possible which improves when given inhaled treatment.

• #34 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 most up to date diagnostic rules, written by the European Respiratory Society (ERS), say that someone with EGPA should have: […] Blood tests will show high levels of Eosinophil white blood cells in patients with active EGPA. […] People with active EGPA may have patches of vasculitic damage where eosinophils invade and damage the lung tissue (infiltrates) that may be visible on a chest x-ray. […] All patients diagnosed with EGPA have asthma, and this will almost always be shown through spirometry (breathing tests) that show an inability to breathe out a quickly as should be possible which improves when given inhaled treatment.

• #35 Churg Strauss Syndrome – Vasculitis UK

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

  If biopsies (tissue samples) are taken, these may show high numbers of eosinophils, collections of immune cells (known as a granuloma), and damage to blood vessels cause by immune cells (vasculitis). […] Camera tests may be performed to wash out areas of the lung. […] People with EGPA can have damage to their heart, and so a heart tracing (electrocardiogram ECG) and/or ultrasound test (echocardiogram) are often performed.

• #36 Churg Strauss Syndrome – Vasculitis UK

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

  If biopsies (tissue samples) are taken, these may show high numbers of eosinophils, collections of immune cells (known as a granuloma), and damage to blood vessels cause by immune cells (vasculitis). […] Camera tests may be performed to wash out areas of the lung. […] People with EGPA can have damage to their heart, and so a heart tracing (electrocardiogram ECG) and/or ultrasound test (echocardiogram) are often performed.

• #37 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

  Antineutrophil cytoplasmic antibody (ANCA), Chest X-ray, CT scan, Biopsy of an affected tissue, such as lung, skin, or nerve, Bronchoalveolar lavage (BAL), Gastrointestinal endoscopy, Urinalysis, Electromyelography (EMG) and nerve conduction studies, Electrocardiogram (ECG) for cardiac manifestations.

• #38 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

  Statement 5: EGPA remission is defined as the absence of clinical signs or symptoms attributable to active disease, including asthma and ENT manifestations. The daily dose of glucocorticoids should also be considered for the definition of remission, and a maximum daily dose of 7.5mg of prednisone can be chosen as the cut-off. (L: 5; G: D) […] Statement 6: Remission-induction treatment should be tailored on the basis of clinical manifestations with prognostic relevance. Organ-threatening manifestations included in the Five-Factor Score (renal insufficiency, proteinuria, cardiomyopathy, gastrointestinal tract and central nervous system involvement) as well as peripheral neuropathy and other rare manifestations (for example, alveolar hemorrhage) should be considered when choosing remission-induction strategies. (L: 2b; G: B)

• #39 How You Get a Diagnosis of Churg-Strauss Syndrome

  https://www.webmd.com/lung/churg-strauss-diagnosis

  Your doctor may order them to measure the number of eosinophils in your blood, or to check for signs of inflammation in your body. High eosinophil levels can mean that you have Churg-Strauss syndrome or asthma. […] A blood test called ANCA looks for these autoantibodies. Only about 30% to 50% of people with Churg-Strauss syndrome will have a positive ANCA test, so it alone can’t confirm the diagnosis. […] If other tests suggest that you might have Churg-Strauss syndrome, your doctor will confirm the results with a biopsy. The doctor removes a small piece of tissue from your lungs, skin, or other organs. Then a lab checks the sample under a microscope for signs of inflammation or damage. […] Biopsy results in people with Churg-Strauss syndrome often show: High numbers of eosinophils, Clusters of immune cells called a granuloma, Blood vessel damage caused by immune cells, called vasculitis.

• #40 Churg-Strauss Disease Workup: Approach Considerations, Laboratory Studies, Imaging Studies

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

  Biopsy specimens of skin, hilar lymph nodes, lung parenchyma, or peripheral nerve demonstrate the characteristic vasculitis. Kidney biopsy sample may show segmental necrotizing glomerulonephritis with crescent formation, possibly including highly characteristic eosinophilic infiltration of the renal interstitium. […] Biopsy results in Churg-Strauss disease (CSD), now known as eosinophilic granulomatosis with polyangiitis (EGPA), may demonstrate eosinophilic vasculitis, especially involving the outer zone of the adventitia of medium (10150 m) to small (3050 m) arteries. However, it may be found that infiltrating cells are predominantly lymphocytic while eosinophils may be less prominent or even rare.

• #41 A case of Churg-Strauss syndrome misdiagnosed as dermatitis – Wang – Journal of Xiangya Medicine

  https://jxym.amegroups.org/article/view/4047/html

  Skin biopsy is helpful in the diagnosis and often occurs the typical microscopic performance. Typical pathology of CSS is extensive vascular necrosis, many eosinophils around the cutaneous vessels, and extravascular granuloma formation, and our pathology was completely consistent with the typical pathology performance.

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

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

  The distinction between early-phase and vasculitic-phase disease may be subtle. […] However, the classic histologic hallmarks of the vasculitic phase are (1) an eosinophil rich necrotizing vasculitis involving primarily small arteries, arterioles, venules, and veins and (2) necrotizing granulomas centered on necrotic eosinophils. […] What is not widely appreciated is how infrequently all of the classic findings are actually found in modern biopsy material; in a recent series of 23 patients, Reid et al. were able to show the original Churg and Strauss criteria in only 4! […] 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.

• #43 What Is Churg-Strauss Syndrome?

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

  Pulmonary Function Test (PFT) – PFT shows lung (airflow) obstruction. […] CT (Computed Tomography) of the Chest and Sinus – CT of the chest and sinus shows thickening of the nasal membrane and sinus walls, lung infiltrates, bronchial wall thickening, […] Sural Nerve Biopsy – Axonal degeneration is present indicative of peripheral neuropathy. Sural nerve biopsy is regarded as the gold standard for Churg-Strauss diagnosis.

• #44 Churg-Strauss Disease Workup: Approach Considerations, Laboratory Studies, Imaging Studies

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

  Biopsy specimens of skin, hilar lymph nodes, lung parenchyma, or peripheral nerve demonstrate the characteristic vasculitis. Kidney biopsy sample may show segmental necrotizing glomerulonephritis with crescent formation, possibly including highly characteristic eosinophilic infiltration of the renal interstitium. […] Biopsy results in Churg-Strauss disease (CSD), now known as eosinophilic granulomatosis with polyangiitis (EGPA), may demonstrate eosinophilic vasculitis, especially involving the outer zone of the adventitia of medium (10150 m) to small (3050 m) arteries. However, it may be found that infiltrating cells are predominantly lymphocytic while eosinophils may be less prominent or even rare.

• #45 How You Get a Diagnosis of Churg-Strauss Syndrome

  https://www.webmd.com/lung/churg-strauss-diagnosis

  Your doctor may order them to measure the number of eosinophils in your blood, or to check for signs of inflammation in your body. High eosinophil levels can mean that you have Churg-Strauss syndrome or asthma. […] A blood test called ANCA looks for these autoantibodies. Only about 30% to 50% of people with Churg-Strauss syndrome will have a positive ANCA test, so it alone can’t confirm the diagnosis. […] If other tests suggest that you might have Churg-Strauss syndrome, your doctor will confirm the results with a biopsy. The doctor removes a small piece of tissue from your lungs, skin, or other organs. Then a lab checks the sample under a microscope for signs of inflammation or damage. […] Biopsy results in people with Churg-Strauss syndrome often show: High numbers of eosinophils, Clusters of immune cells called a granuloma, Blood vessel damage caused by immune cells, called vasculitis.

• #46 Churg Strauss Syndrome – Vasculitis UK

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

  If biopsies (tissue samples) are taken, these may show high numbers of eosinophils, collections of immune cells (known as a granuloma), and damage to blood vessels cause by immune cells (vasculitis). […] Camera tests may be performed to wash out areas of the lung. […] People with EGPA can have damage to their heart, and so a heart tracing (electrocardiogram ECG) and/or ultrasound test (echocardiogram) are often performed.

• #47 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) – Vasculitis Syndromes – Rheumatology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.16.9.4.

  The 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology criteria should be applied to classify EGPA but only when a diagnosis of small-sized or medium-sized vasculitis has been made. […] A score 6 is required for the diagnosis of EGPA. […] Differential diagnosis should include hypereosinophilic syndrome (HES), drug reactions (drug reaction with eosinophilia and systemic symptoms [DRESS]), parasitic or fungal infections, other systemic vasculitides, eosinophilic asthma/pneumonia, cancer, and lymphoma.

• #48 Eosinophilic Granulomatosis with Polyangiitis (EGPA) – Rare Awareness Rare Education

  https://rareportal.org.au/rare-disease/eosinophilic-granulomatosis-with-polyangiitis-egpa/

  Eosinophilic granulomatosis with polyangiitis (EGPA) may be diagnosed based on physical examination, extensive medical history, blood tests to check for high levels of eosinophils (a type of white blood cell) and MPO-ANCA antibodies, and imaging scans. […] A tissue sample (biopsy) may also be taken to look for inflammation and narrowing of blood vessels (vasculitis/angiitis) and the presence of eosinophils (white blood cells). The presence of vasculitis and eosinophils can help to distinguish EGPA from other diseases that have similar signs and symptoms. […] Recommendations for diagnosis of EGPA that were developed by a panel of European experts can be found at Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis. […] Differential diagnosis (to rule out other conditions) includes eosinophilic lung diseases (such as eosinophilic pneumonia, allergic bronchopulmonary aspergillosis), other types of small and medium vessel vasculitis (such as granulomatosis with polyangiitis, polyarteritis nodosa) fungal and parasitic infections, and malignancy. […] Please speak to your medical team to learn more about the available diagnostic pathways for EGPA.

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

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

  Most pathologists assume that a diagnosis of Churg-Strauss syndrome (CSS) requires the finding of necrotizing vasculitis accompanied by granulomas with eosinophilic necrosis in the setting of asthma and eosinophilia. However, recent data indicate that this definition is too narrow and that adherence to it leads to cases of CSS being missed. […] CSS has an early, prevasculitic phase that is characterized by tissue infiltration by eosinophils without overt vasculitis. […] Tissue infiltration may take the form of a simple eosinophilia in any organ, and a fine-needle aspirate showing only eosinophils may suffice for the diagnosis in this situation. […] Even in the vasculitic phase of CSS, many cases do not show a necrotizing vasculitis but often only an apparently nondestructive infiltration of vessel walls by eosinophils.

• #50 Churg-Strauss Syndrome with Orbital Inflammation

  https://webeye.ophth.uiowa.edu/eyeforum/cases/119-churg-strauss-syndrome.htm

  Churg-Strauss syndrome (CSS) was first described by Churg and Strauss in 1951 as a small and medium-vessel vasculitis characterized by asthma, hypereosinophilia and multi-system vasculitis (Sehgal, 1995). […] The patient’s constellation of clinical and laboratory findings satisfied the American College of Rheumatology criteria for Churg-Strauss Syndrome (CSS) (Table 1) Specifically, his asthma, peripheral eosinophilia, paranasal sinusitis and histologic proof of eosinophilic perivasculitis made CSS the leading diagnosis for this patient. […] Establishing a diagnosis can be difficult given the phasic nature and the often prolonged and sequential development of clinical and pathological features. CSS is distinguished from other granulomatous, vasculitic and eosinophilic syndromes by a clinical history that includes asthma, eosinophilia and rhinosinusitis, as well as other characteristic signs and symptoms (Table 2). Establishing a diagnosis depends on the presence of a constellation of findings, rather than any single feature.

• #51 Differential Diagnosis of Churg-Strauss Syndrome with Tingling Sensation and Weakness of the Lower Extremities

  https://brieflands.com/articles/aapm-84179

  The CSS usually manifests in three stages. The early stage is marked by airway inflammation. […] Early diagnosis and treatment will prevent organ damage and mortality. […] Although classified as vasculitis, ANCA positivity is observed in only 40% to 60% of patients. […] The five-year survival rate for people with Churg-Strauss angiitis is estimated to be 66% to 100%. However, the morbidity is considerable when assessed by health-related quality-of-life measures. […] Therefore, early differential diagnosis and adequate treatment are important for patients with CSS.

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

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

  Both the ACR and the Lanham criteria also emphasize allergic rhinitis as a part of CSS. […] CSS is associated with a positive ANCA in some reports in as many as 70% of cases; this is usually p- (myeloperoxidase) ANCA, although c-ANCA positive patients have also been reported, and sometimes the proportion of positive cases is much smaller. […] There appears to be a consensus that a properly performed ANCA test, which must include an ELISA to show proteinase 3 or myeloperoxidase specificity, provides strong support for a diagnosis of CSS, even if overt vasculitis cannot be found. […] The characteristic feature of the early phase of CSS is extravascular tissue infiltration by eosinophils, and this condition is, in my experience, underrecognized by pathologists, not in the sense of morphology, but in the sense of indicating to the clinician the significance of eosinophils as a confirmatory diagnostic finding.

• #53 SciELO Brazil – Cutaneous manifestations of Churg-Strauss syndrome: key to diagnosis* Cutaneous manifestations of Churg-Strauss syndrome: key to diagnosis*

  https://www.scielo.br/j/abd/a/3sQNtqTK6mLdD9SSDBhsV7F/?lang=en

  Churg-Strauss syndrome is a rare systemic vasculitis characterized by asthma and other allergy symptoms as well as eosinophilia and necrotizing vasculitis involving small and medium-sized vessels. […] The picture of asthma, fever, multiple mononeuropathy and eosinophilia, associated to sparse papular-purpuric lesions lead to the suspicion of CSS. […] A biopsy was taken from the patients left foot, and the histology shown in Figure 4, showed multiple changes of small vessels with fibrin deposition and endothelial swelling, besides leukocytoclasis and malformed granulomas with multiple conglomerates of eosinophils. Such features supported the hypothesis of CSS. […] CSS diagnostic criteria defined by the American College of Rheumatology (ACR) are described in Chart 1. […] Despite the mild cutaneous manifestations of the patient, there were four undisputable diagnostic criteria present: history of asthma, eosinophilia, neuropathy and vascular eosinophilic infiltrate on the histology. Besides, p-ANCA was positive, making it possible to diagnose the third phase of CSS. […] Recognition of these lesions by the dermatologist was essential for the clinical suspicion and confirmation of diagnosis, that allowed adequate treatment, reducing morbidity and contributing for the prevention of irreversible lesions in vital organs.

• #54 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

  Statement 5: EGPA remission is defined as the absence of clinical signs or symptoms attributable to active disease, including asthma and ENT manifestations. The daily dose of glucocorticoids should also be considered for the definition of remission, and a maximum daily dose of 7.5mg of prednisone can be chosen as the cut-off. (L: 5; G: D) […] Statement 6: Remission-induction treatment should be tailored on the basis of clinical manifestations with prognostic relevance. Organ-threatening manifestations included in the Five-Factor Score (renal insufficiency, proteinuria, cardiomyopathy, gastrointestinal tract and central nervous system involvement) as well as peripheral neuropathy and other rare manifestations (for example, alveolar hemorrhage) should be considered when choosing remission-induction strategies. (L: 2b; G: B)

• #55 Churg-Strauss syndrome – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/churg-strauss-syndrome/diagnosis-treatment/drc-20353765

  To diagnose Churg-Strauss syndrome, doctors usually request several types of tests, including: […] A blood test can detect certain antibodies in your blood that can suggest, but not confirm, a diagnosis of Churg-Strauss syndrome. […] X-rays and CT scans can reveal abnormalities in your lungs and sinuses. […] If other tests suggest Churg-Strauss syndrome, you might have a small sample of tissue removed for examination under a microscope. […] If you have signs and symptoms common to Churg-Strauss syndrome, make an appointment with your doctor. Early diagnosis and treatment significantly improve the outlook of this condition. […] You may be referred to a doctor who specializes in disorders that cause blood vessel inflammation (vasculitis), such as a rheumatologist or immunologist. […] Basic questions to ask your doctor might include: What diagnostic tests do I need? […] A doctor who sees you for possible Churg-Strauss syndrome is likely to ask you questions, such as: Have your symptoms, especially asthma-related, worsened over time?

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

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

  Churg-Strauss syndrome is rare, and it’s more likely that these symptoms have some other cause. But it’s important that your doctor evaluate them. Early diagnosis and treatment improve the chances of a good outcome. […] Churg-Strauss syndrome can affect many organs, including the lungs, sinuses, skin, gastrointestinal system, kidneys, muscles, joints and heart. Without treatment, the disease can be fatal. […] Diagnosis treatment

• #57 Churg-Strauss syndrome – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/churg-strauss-syndrome/diagnosis-treatment/drc-20353765

  To diagnose Churg-Strauss syndrome, doctors usually request several types of tests, including: […] A blood test can detect certain antibodies in your blood that can suggest, but not confirm, a diagnosis of Churg-Strauss syndrome. […] X-rays and CT scans can reveal abnormalities in your lungs and sinuses. […] If other tests suggest Churg-Strauss syndrome, you might have a small sample of tissue removed for examination under a microscope. […] If you have signs and symptoms common to Churg-Strauss syndrome, make an appointment with your doctor. Early diagnosis and treatment significantly improve the outlook of this condition. […] You may be referred to a doctor who specializes in disorders that cause blood vessel inflammation (vasculitis), such as a rheumatologist or immunologist. […] Basic questions to ask your doctor might include: What diagnostic tests do I need? […] A doctor who sees you for possible Churg-Strauss syndrome is likely to ask you questions, such as: Have your symptoms, especially asthma-related, worsened over time?

• #58 Diagnosing Eosinophilic Granulomatosis: Symptoms, Causes & Treatment – The Kingsley Clinic

  https://thekingsleyclinic.com/resources/diagnosing-eosinophilic-granulomatosis-symptoms-causes-treatment/

  Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare autoimmune condition characterized by inflammation of small to medium-sized blood vessels. […] Early diagnosis and treatment are essential to prevent severe complications, such as heart failure, kidney damage, or nerve damage. […] A diagnosis of Churg-Strauss syndrome is equivalent to a diagnosis of EGPA. […] Diagnostic tests can help distinguish these conditions. GPA is often associated with anti-neutrophil cytoplasmic antibodies (ANCAs), particularly c-ANCA, while EGPA is linked to elevated eosinophil levels (a type of white blood cell). […] Blood tests can help distinguish EGPA from other forms of vasculitis. EGPA is characterized by high eosinophil levels and positive ANCA tests, particularly p-ANCA. A biopsy of affected tissue can confirm the diagnosis by identifying specific patterns of inflammation. […] If you’re experiencing symptoms of eosinophilic granulomatosis with polyangiitis or need assistance managing your condition, our telemedicine practice is here to help.

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

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

  Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, belongs to a group of diseases characterized by necrotizing vasculitis affecting small- and medium-sized systemic blood vessels, also known as antineutrophil cytoplasmic autoantibody (ANCA)associated vasculitis (AAV). […] Although EGPA is classified as an ANCA-associated vasculitis, ANCA is positive in only 30% to 40% of cases, making diagnosis challenging. […] Clinical recognition of multisystem involvement, especially in patients with eosinophilic asthma, is key to diagnosis. […] The management of EGPA also differs from other AAVs, requiring tailored treatment strategies. […] The scoring system developed by the ACR and European Alliance of Associations for Rheumatology (ACR-EULAR) in 2022 for diagnosing EGPA includes: Maximum eosinophil count of 1109 cells/L or higher (+5), Obstructive airway disease (+3), Nasal polyps (+3), ANCA or anti-proteinase 3-ANCA positivity (3), Extravascular eosinophilic predominant inflammation (+2), Mononeuritis multiplex or motor neuropathy not due to radiculopathy (+1), Hematuria (1).

• #60 Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-us/942

  Investigations to consider include flow cytometry of peripheral blood, molecular testing for FIP1L1/PDGFR alpha mutation, serum IgE, serum-specific IgE and IgG to Aspergillus fumigatus, stool culture for ova and parasites, Toxocara serology, CT chest, electromyogram, bronchoscopy with bronchoalveolar lavage (BAL), biopsy of affected tissue, lower extremity ultrasound, CT angiography, bone marrow biopsy, thiopurine methyltransferase level, and HIV testing. […] Emerging tests include cardiac MRI and eotaxin-3.

• #61 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

• #62 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

• #63 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

• #64 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

  Statement 10: Relapses should be treated according to type (systemic versus respiratory) and severity. For severe systemic relapses, we recommend using rituximab or cyclophosphamide with glucocorticoids. For non-severe systemic and respiratory relapses, we recommend increasing the dose of glucocorticoids and/or adding mepolizumab. (L: 2b; G: C) […] Statement 11: Refractory EGPA is defined as unchanged or increased disease activity after 4 weeks of appropriate remission-induction therapy. The persistence or worsening of systemic manifestations should be distinguished from that of respiratory manifestations. (L: 5; G: D) […] Statement 12: The IL-5 inhibitor mepolizumab in combination with glucocorticoids is recommended to induce remission in patients with relapsing-refractory EGPA without organ- or life-threatening manifestations. Mepolizumab can also be used for remission maintenance, particularly in patients requiring a daily prednisone dose 7.5mg for the control of their respiratory manifestations. (L: 2b; G: B)

• #65 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

• #66 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

• #67 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

• #68 How You Get a Diagnosis of Churg-Strauss Syndrome

  https://www.webmd.com/lung/churg-strauss-diagnosis

  It can take a few steps to find out whether you have Churg-Strauss syndrome. This rare disease is hard to diagnose based on its typical symptoms like fever, asthma, appetite loss, and tiredness. The trouble is that many conditions can cause these same symptoms. […] There also isn’t a single test to diagnose Churg-Strauss syndrome. Your doctor will look at many different things, including your symptoms, a physical exam, and the results of your lab and imaging tests. […] The American College of Rheumatology has set up six „criteria” — or standards — for diagnosing Churg-Strauss syndrome. These are signs that most people with the condition have. […] Your doctor will diagnose you with Churg-Strauss syndrome if you meet four of these six criteria: […] Doctors diagnose Churg-Strauss syndrome using one or more of these tests:

• #69 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

  Statement 5: EGPA remission is defined as the absence of clinical signs or symptoms attributable to active disease, including asthma and ENT manifestations. The daily dose of glucocorticoids should also be considered for the definition of remission, and a maximum daily dose of 7.5mg of prednisone can be chosen as the cut-off. (L: 5; G: D) […] Statement 6: Remission-induction treatment should be tailored on the basis of clinical manifestations with prognostic relevance. Organ-threatening manifestations included in the Five-Factor Score (renal insufficiency, proteinuria, cardiomyopathy, gastrointestinal tract and central nervous system involvement) as well as peripheral neuropathy and other rare manifestations (for example, alveolar hemorrhage) should be considered when choosing remission-induction strategies. (L: 2b; G: B)

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

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

  Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, belongs to a group of diseases characterized by necrotizing vasculitis affecting small- and medium-sized systemic blood vessels, also known as antineutrophil cytoplasmic autoantibody (ANCA)associated vasculitis (AAV). […] Although EGPA is classified as an ANCA-associated vasculitis, ANCA is positive in only 30% to 40% of cases, making diagnosis challenging. […] Clinical recognition of multisystem involvement, especially in patients with eosinophilic asthma, is key to diagnosis. […] The management of EGPA also differs from other AAVs, requiring tailored treatment strategies. […] The scoring system developed by the ACR and European Alliance of Associations for Rheumatology (ACR-EULAR) in 2022 for diagnosing EGPA includes: Maximum eosinophil count of 1109 cells/L or higher (+5), Obstructive airway disease (+3), Nasal polyps (+3), ANCA or anti-proteinase 3-ANCA positivity (3), Extravascular eosinophilic predominant inflammation (+2), Mononeuritis multiplex or motor neuropathy not due to radiculopathy (+1), Hematuria (1).

• #71 Identifying EGPA | NUCALA (mepolizumab) for HCPs

  https://nucalahcp.com/egpa/egpa-patient-types/identifying-egpa/

  A biopsy containing a blood vessel with extravascular eosinophils supports a diagnosis in the context of other clinical manifestations of EGPA. […] With no accepted diagnostic criteria for EGPA, its diagnosis is based on the clinical and laboratory or biopsy criteria shown on screen. In the 2022 ACR and EULAR classification criteria, a cumulative score of greater than or equal to 6 points yields an 85% sensitivity and 99% specificity for EGPA. These criteria are meant for classification and not diagnostic purposes.

• #72 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

  Statement 3: The diagnostic evaluation of patients with suspected EGPA should always be multidisciplinary; it should rule out other eosinophilic and vasculitic disorders and investigate the main disease complications, particularly heart, respiratory, skin, renal and nervous system involvement, along with ANCA and eosinophilia. Biopsy is recommended when feasible, but is not essential to make the diagnosis of EGPA. (L: 3b; G: C) […] Statement 4: ANCA testing should be performed in all patients with suspected EGPA. ANCA are detectable in 30-40% of patients with EGPA, most of whom test positive for MPO-ANCA. MPO-ANCA-positive patients frequently show vasculitis features, such as glomerulonephritis, neuropathy and purpura, whereas ANCA-negative patients more frequently manifest cardiomyopathy and lung involvement. (L: 2a; G: B)

• #73 Churg-Strauss syndrome (CSS) or Eosinophilic granulomatosis (EGPA) – Autoimmune Association

  https://autoimmune.org/disease-information/churg-strauss/

  Churg-Strauss syndrome, also known as allergic granulomatosis or eosinophilic granulomatosis with polyangiitis (EGPA) is an autoimmune disorder characterized by accumulated antibodies, inflammation of blood vessels, and abnormal clustering of white blood cells. […] Diagnosis is difficult because early symptoms mimic common flu. […] Prompt diagnosis and treatment (with corticosteroids) increase a patient’s chances of resuming a normal life.

• #74 Churg-Strauss syndrome – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/churg-strauss-syndrome/diagnosis-treatment/drc-20353765

  To diagnose Churg-Strauss syndrome, doctors usually request several types of tests, including: […] A blood test can detect certain antibodies in your blood that can suggest, but not confirm, a diagnosis of Churg-Strauss syndrome. […] X-rays and CT scans can reveal abnormalities in your lungs and sinuses. […] If other tests suggest Churg-Strauss syndrome, you might have a small sample of tissue removed for examination under a microscope. […] If you have signs and symptoms common to Churg-Strauss syndrome, make an appointment with your doctor. Early diagnosis and treatment significantly improve the outlook of this condition. […] You may be referred to a doctor who specializes in disorders that cause blood vessel inflammation (vasculitis), such as a rheumatologist or immunologist. […] Basic questions to ask your doctor might include: What diagnostic tests do I need? […] A doctor who sees you for possible Churg-Strauss syndrome is likely to ask you questions, such as: Have your symptoms, especially asthma-related, worsened over time?

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