Materiały źródłowe

• #1 Churg-Strauss syndrome from an orthopaedic perspective | HKMJ

  https://www.hkmj.org/abstracts/v21n6/565.htm

  Churg-Strauss syndrome, which has been frequently described by physicians in the literature, is a small and medium-sized vessel systemic vasculitis typically associated with asthma, lung infiltrates, and hypereosinophilia. […] Churg-Strauss syndrome (CSS) is a small and medium-sized vessel vasculitis that can affect different organs. […] This case illustrates that common symptoms such as pain and numbness, frequently encountered when treating orthopaedic patients, may not be necessarily due to an orthopaedic problem. […] The American College of Rheumatology has recommended that diagnosis of the syndrome is considered when four of the following features are present: (1) asthma, (2) eosinophils constituting more than 10% of the white cell count, (3) neuropathy, (4) non-fixed pulmonary infiltrates on radiography, (5) extravascular granulomas, and (6) abnormalities of the paranasal sinuses.

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

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

  The incidence and prevalence of EGPA is approximately 1.7 and 14.25 cases per million persons, respectively, making EGPA the least common of all the ANCA vasculitides. […] The country with the highest overall incidence is the United States, at 4 cases per million persons. The prevalence is highest in Norway, with 30.4 cases per million persons, and within Europe, Norway has the highest incidence at 2.5 cases per million persons. […] The prevalence of EGPA is equal in men and women. Onset occurs most often between the ages of 40-60 years, with the mean age at diagnosis being 50 years.

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

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

  The estimated prevalence of Churg-Strauss disease (CSD), now known as eosinophilic granulomatosis with polyangiitis (EGPA), is 10.7 to 14 per million adults worldwide. There is no gender difference in the incidence of the disease. The age distribution is wide with reports of patients as young as 4 years and as old as 74 years; median age at presentation is 40 years old. […] The chief causes of mortality related to EGPA are severe asthma, cardiopulmonary failure, or gastrointestinal complications. In general, the long-term outcome of EGPA does not differ greatly from that of PAN. In addition to overall severity of EGPA, both myocardial disease and severe gastrointestinal disease were found by Guillevin et al to be independent predictors of poor prognosis. […] Across the years there has been a constant decrease in the mortality rate of patients who suffered from EGPA. Most (85%) of the index cases reported by Churg and Strauss in 1951 had died from their illness. The development of effective strategies for treatment in ensuing decades (particularly oral corticosteroids) has improved survival considerably, though the disease remains a serious one. To some extent, improved survival may relate to recognition of milder cases.

• #1 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 incidence of EGPA ranges between 0.5 and 4.2 cases per million people per year and its prevalence between 10 and 14 cases per million inhabitants globally. The frequency of the disease is comparable in men and women, and the mean age at diagnosis is ~50 years. Paediatric cases are extremely rare. […] Given the rarity of EGPA, its heterogeneous clinical presentation and the clinical overlap with other vasculitic or eosinophilic disorders, the diagnosis of EGPA is often challenging. Multiple disciplines are involved in the care of patients, which dictates an integrated and collaborative approach. To date, no systematically developed, evidence-based guidelines have been specifically dedicated to the diagnosis and management of EGPA, and current practice is based mainly on the 2015 recommendations for EGPA published by a consensus task force and on the 2016 EULAR/European Renal Association (ERA)-European Dialysis and Transplant (EDTA) Association recommendations for ANCA-associated vasculitis (AAV). More recently, the 2021 ACR-Vasculitis Foundation guidelines for the management of AAV were developed. The 2016 and 2021 guidelines, however, cover all forms of AAV and were not developed specifically for EGPA.

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

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

  The estimated prevalence of the disease is approximately 1 to 3 per million adults worldwide. The mean age of onset, as reported, ranges between 38 and 54, with a median of 40. However, cases have also been reported in extremes of age, from as young as 4 to as high as 74. Gender prevalences are similar between males and females. […] In contrast to certain small vessel vasculitides, such as immunoglobulin A (IgA) vasculitis and Kawasaki disease, with a preponderance in childhood, AAV is rare in children. However, regardless of age, both adult and childhood EGPA present with highly elevated IgE and eosinophil levels. About 40% of adults with EGPA have a positive ANCA, whereas 25% of children are seropositive for this. […] Clinically, childhood EGPA has a higher incidence of cardiomyopathy and pneumonic infiltrates, with a lower occurrence of mononeuritis multiplex. The increased prevalence of cardiac disease contributes to higher mortality in childhood EGPA, similar to that seen in adult cases.

• #1 Eosinophilic Granulomatosis Polyangiitis (EGPA) – EyeWiki

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

  Epidemiology […] EGPA is not as common as the other AAVs with estimated annual incidence and prevalence at 4 and 18 cases per million, respectively. However, the annual incidence increases to about 67 cases per million among asthmatic patients. The average age of diagnosis ranges between 38 54 years and there is no notable ethnic, familial, or gender predisposition. […] […] The general approach to EGPA treatment is like other AAV (e.g., GPA and MPA) treatments. Induction immunosuppressive therapy followed by remission and maintenance therapy is a common algorithm for EGPA. The severity of EGPA is scored based on a five factor score (FFS), an algorithm developed by the French Vasculitis Study Group. FFS separates severe from non-severe disease based on a scoring system that accounts for factors such as age, creatinine, and organ involvement.

• #1 Incidence, Prevalence, and Mortality of Eosinophilic Granulomatosis With Polyangiitis in Korea: A Nationwide Population-Based Study

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10973633/

  Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of vasculitis with multiorgan involvement. The incidence and prevalence of EGPA vary geographically and ethnically. This study investigated the incidence, prevalence, and mortality of EGPA in a nationwide population-based cohort in Korea. A total of 843 patients with EGPA were identified. The mean annual standardized incidence between 2007 and 2018 was 1.2 (per 1,000,000 individuals). The incidence of EGPA has increased from 1.1 (per 1,000,000 individuals) in 2007 to 1.6 (per 1,000,000 individuals) in 2017. The standardized prevalence of EGPA has increased from 1.1(per 1,000,000 individuals) in 2007 to 11.2 (per 1,000,000 individuals) in 2018. The incidence and prevalence of EGPA were higher in women than in men. The standardized mortality rate was 1.61 (95% confidence interval [CI], 1.34-1.93) in total population, 1.59 (95% CI, 1.23-2.02) in males, and 1.63 (95% CI, 1.22-2.13) in females. The incidence of EGPA has increased over the past decade. Incidence and prevalence rates were higher in females than in males. The overall mortality rate associated with EGPA was higher than that in the general population. This is the first population-based study on the incidence, prevalence, and mortality of EGPA in Korea using a nationwide claims database. The annual average standardized incidence from 2007 to 2018 and the standardized prevalence in 2018 per 1,000,000 individuals were 1.2 and 11.2, respectively, with a significant increase observed since 2014. Incidence and prevalence rates were higher in women than in men. The overall mortality rate of patients with EGPA was higher than that of the general population.

• #1 Eosinophilic granulomatosis with polyangiitis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/eosinophilic-granulomatosis-with-polyangiitis?lang=us

  The incidence typically peaks in middle age, 30-50 years of age with pediatric cases being rare. The annual incidence rate of around 0.24-1.78 per 100,000. There is no gender predilection. […] Eosinophilic granulomatosis with polyangiitis, previously known as Churg-Strauss syndrome, is characterized by asthma, eosinophilia and small vessel necrotizing vasculitis and involves the lungs and other organs.

• #1 Churg-Strauss syndrome epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Churg-Strauss_syndrome_epidemiology_and_demographics

  The incidence of eosinophilic granulomatosis with polyangiitis ranges from 2.5 to 16.6 per 100,000 individuals. Prevalence ranges from 2 to 16 per 100,000 individuals. […] The prevalence of eosinophilic granulomatosis with polyangiitis ranges from 2 to 16 per 100,000 individuals. […] The incidence of eosinophilic granulomatosis with polyangiitis range from 2.5 to 16.6 per 100,000 individuals. […] The mean age at diagnosis of eosinophilic granulomatosis with polyangiitis is around 45-50 years. […] In general, there is no gender predilection to eosinophilic granulomatosis with polyangiitis in western nations. In Japan, female predominance has been noted. […] In general, there is no racial predominance to eosinophilic granulomatosis with polyangiitis.

• #1 Churg-Strauss Syndrome with Orbital Inflammation

  http://eyerounds.org/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). CSS is a rare disorder with an incidence of 1.3 to 6.8 cases per 1 million patients per year (Scott, 2000). The mean age of presentation is approximately 50 years, and there appears to be a slight male predominance. […] Epidemiology: Mean age 50 years old; Possible slight male predominance; 1.3- 6.8 cases per 1 million per year.

• #1 Churg-Strauss syndrome – WikiLectures

  https://www.wikilectures.eu/w/Churg-Strauss_syndrome

  The incidence of Churg-Strauss syndrome is reported as 2,4/1 000 000. The disease manifests between the ages of 14 and 75, the most common occurrence being in the 4th and 5th decade. Men are slightly more likely to be affected by CSS.[1] […] Before corticosteroids were introduced, half of patients died within 3 months of the first signs of vasculitis and less than 5% of patients survived for 5 years. Currently, more than 75% survive for five years thanks to combination treatment.[1] […] As this is a very rare disease, patients with CSS are clearly indicated for care in specialized centers and under conditions of interdisciplinary cooperation.

• #1 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/

  CSS diagnostic criteria defined by the American College of Rheumatology (ACR) are described in Chart 1. […] CSS prognosis is variable and depends on the initial extension of the disease and the organs affected. Five factors were described that are associated to a higher mortality among patients; which are: creatinine higher than 1.58mg/dL, proteinuria higher than 1 g/day, gastrointestinal involvement, central nervous system involvement and cardiomyopathy. […] Cutaneous manifestations of CSS are one type of clinical presentation for this condition. 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.

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

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

  Estimated prevalence of the disease is 10.7 to 14 per million adults worldwide. The mean age of onset, is between 38 to 54 years, with a median of 40. There is no gender difference in incidence. […] Churg-Strauss vasculitis is a rare disease, but there is a possibility of misdiagnosis and missed diagnoses. However, cardiac involvement is present in more than 50% of patients with EGPA, which is the major cause of morbidity and mortality. […] Cardiac involvement is associated with a poor prognosis, and therefore early diagnosis and institution of treatment is imperative. […] In a recent article by Pakbaz et al, they collected the clinical data of 62 patients suffering from EGPA with cardiac involvement from the literature. CMR was done for 29 (46.8%) patients, and it was abnormal in all; thus, it is probably the most sensitive method for detecting cardiac involvement in patients with EGPA. […] Therefore, CMR is the considered the gold standard noninvasive diagnostic test for early diagnosis and rapid institution of therapy.

• #1 Epidemiology of eosinophilic granulomatosis with polyangiitis » Institute for rare diseases

  https://www.raredis.org/archives/24206?lang=en

  Eosinophilic granulomatosis with polyangiitis (EGPA), until recently called Churg-Strauss syndrome is a systemic necrotizing vasculitis with multiple organ involvement, most commonly manifested by asthma, eosinophilia and necrotizing extravasal granulomas. […] The lack of a unified approach in the application of these criteria, as well as the overlap of clinical symptoms with other primary systemic vasculitides, make it difficult to study EGPA epidemiology. […] This publication aims to analyze epidemiological data on EGPA and to provide an assessment of the incidence and prevalence of this disease in Bulgaria.

• #1 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 […] EGPA is one of the rarest forms of vasculitis and primarily affects the small blood vessels. […] 11-14 Cases per Million Worldwide […] Number of US cases unknown […] Average age is 35-50, but can occur at any age […] Rare in children […] Ethnic prevalence unknown. […] EGPA is considered extremely rare. […] The cause of EGPA is not fully understood. […] Researchers believe an infection may set the inflammatory process in motion. […] Regular doctor visits and ongoing monitoring of lab and imaging tests are important in detecting relapses early. […] The majority of EGPA patients respond well to treatment. […] Because relapses are common with EGPA, follow-up medical care is essential.

• #1 Peripheral Neuropathy as Initial Manifestation of Churg-Strauss Syndrome

  https://www.clinmedjournals.org/articles/iaim/international-archives-of-internal-medicine-iaim-3-021.php?jid=iaim

  Churg-Strauss syndrome is a systemic disorder characterized by asthma, hypereosinophilia, and systemic vasculitis and frequently involves peripheral nerves and skin. […] EGPA have annual incidence rates of 0.5-3.7 per million in Europe. […] The relative rarity and non-specific presentation of the ANCA-associated vasculitides pose diagnostic challenges and often result in a significant diagnostic delay of more than 6 months in a third of patients. […] Neurological symptoms can be seen in 60-70% of patients with EGPA, secondary only to pulmonary manifestations in the overall frequency of organ involvement. […] An early recognition of this entity is the key in leading to timely treatment and positive outcome. […] The diagnosis of polyneuropathy is based on clinical and electrophysiologic studies, but precise histology, immunohistochemistry and morphometric studies of the peripheral nerve biopsy may be decisive to make diagnosis. […] Churg-Strauss syndrome frequently presents polyneuropathy as a complication; since its remission depends on immunosuppression therapy, it is important to recognize it at an early stage.

• #1 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

  The clinical manifestations of eosinophilic granulomatosis with polyangiitis (EGPA) are quite heterogeneous and their frequencies differ on the basis of anti-neutrophil cytoplasmic antibody (ANCA) status. Specifically, vasculitic features (for example, glomerulonephritis, peripheral neuropathy and purpura) occur more often in ANCA-positive patients, whereas eosinophilic features (such as cardiac involvement and gastroenteritis) are more frequent in ANCA-negative patients. […] 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.

• #1 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

  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. […] The 16 statements are reported and discussed below and in Table 2, and the overarching principles are reported in Box 1. The level of evidence (L) and grade (G) are reported at the end of each statement.

• #1 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

  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. […] The clinical phenotype of EGPA is quite heterogeneous and the diagnosis is not always straightforward. Anti-neutrophil cytoplasmic antibodies (ANCA), usually against myeloperoxidase (MPO), are detectable in ~40% of the cases and are associated with a different frequency of clinical manifestations: features of vasculitis, particularly glomerulonephritis, peripheral neuropathy and purpura, occur more often in ANCA-positive patients, whereas the so-called eosinophilic features such as cardiac involvement and gastroenteritis are more frequent in ANCA-negative patients.

• #1 Epidemiology of Eosinophilic Granulomatosis with Polyangiitis (EGPA) and Hypereosinophilic Syndrome (HES) in Germany: A Claims Database Study – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/epidemiology-of-eosinophilic-granulomatosis-with-polyangiitis-egpa-and-hypereosinophilic-syndrome-hes-in-germany-a-claims-database-study/

  Epidemiology and Public Health Poster III […] Robust data on the epidemiology and treatment of HES and EGPA are scarce. […] Projected to the total German population, prevalence of EGPA and HES in 2019 were 3.89 and 10.44, while incidence proportions were 0.75 patients and 3.95 per 100,000 population, respectively. […] Although both diseases are rare, prevalence increased continuously in the years 2015-2019. […] EGPA and HES were diagnosed more frequently than it was estimated by previous self-reporting registry-based studies. […] The findings indicate that in the past epidemiological data of EGPA and HES may have been underestimated but may also reflect improved diagnostic methods and disease recognition.

• #1

  https://link.springer.com/article/10.1007/s11926-008-0074-x

  Churg-Strauss syndrome (CSS) is a rare necrotizing small-vessel vasculitis associated with eosinophil-rich granulomatous inflammation of tissues and vessels and is also associated with asthma and eosinophilia. […] Epidemiologic studies continue to show that CSS is the rarest of the necrotizing small-vessel vasculitides. […] However, it is not possible to know with any certainty if there has been an increase in incidence. […] There has been an attempt to divide the patients with CSS into an antineutrophil cytoplasmic antibody-positive and cytoplasmic antibody-negative group. […] The role of eosinophils and antineutrophil cytoplasmic antibodies remains poorly defined but provocative. […] Watts R, Lane S, Bentham G, et al.: Epidemiology of systemic vasculitis: a ten-year study in the United Kingdom.

• #1 AAIR :: Allergy, Asthma & Immunology Research

  https://e-aair.org/DOIx.php?id=10.4168/aair.2024.16.2.168

  Eosinophilic granulomatosis with polyangiitis (EGPA), formerly Churg-Strauss Syndrome, is a rare vasculitic disorder of small- to medium-size vessels. The incidence and prevalence of EGPA vary geographically and ethnically. This study investigated the incidence, prevalence, and mortality of EGPA in a nationwide population-based cohort in Korea. The mean annual standardized incidence between 2007 and 2018 was 1.2 (per 1,000,000 individuals). The incidence of EGPA has increased from 1.1 (per 1,000,000 individuals) in 2007 to 1.6 (per 1,000,000 individuals) in 2017. The standardized prevalence of EGPA has increased from 1.1(per 1,000,000 individuals) in 2007 to 11.2 (per 1,000,000 individuals) in 2018. The incidence and prevalence of EGPA were higher in women than in men. The overall mortality rate associated with EGPA was higher than that in the general population. Despite progress in understanding and treating EGPA, its epidemiology remains unclear. The prevalence of EGPA per 1,000,000 population was reported as 10.7 and 17.8 in two previous studies. The annual incidence of EGPA per 1,000,000 population ranges from 0.5 to 3.1. However, most epidemiologic studies were based on patients medical records from local hospitals or information obtained from hospital surveys. This study aimed to investigate the incidence, prevalence, and mortality rates of EGPA in a nationwide population-based cohort. The annual average standardized incidence from 2007 to 2018 and the standardized prevalence in 2018 per 1,000,000 individuals were 1.2 and 11.2, respectively, with a significant increase observed since 2014. Incidence and prevalence rates were higher in women than in men. The overall mortality rate of patients with EGPA was higher than that of the general population. Therefore, active investigation of EGPA for early detection is imperative when patients with asthma and blood eosinophilia present with symptoms affecting other organs to avoid delaying treatment, leading to an increased mortality risk.

• #1 Churg-Strauss Syndrome Market Size & Forecast, 2035

  https://www.imarcgroup.com/churg-strauss-syndrome-market

  The 7 major Churg-Strauss syndrome markets are expected to exhibit a CAGR of 4.37% during 2025-2035. […] The Churg-Strauss syndrome market has been comprehensively analyzed in IMARC’s new report titled „Churg-Strauss Syndrome Market: Epidemiology, Industry Trends, Share, Size, Growth, Opportunity, and Forecast 2025-2035”. […] According to the report, the United States has the largest patient pool for Churg-Strauss syndrome and also represents the largest market for its treatment. […] What is the number of prevalent cases (2019-2035) of Churg-Strauss syndrome across the seven major markets? […] What is the size of the Churg-Strauss syndrome patient pool (2019-2024) across the seven major markets? […] What would be the forecasted patient pool (2025-2035) across the seven major markets? […] What are the key factors driving the epidemiological trend of Churg-Strauss syndrome? […] What will be the growth rate of patients across the seven major markets?

• #2 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. […] CSS is a very rare disease, with an incidence of 17 cases per million per year. […] The disease affects both genders equally and can present at any age, with an average age of 50 at presentation. […] The diagnosis of CSS is generally dependent on histological analysis, although this may not be needed if the patient has eosinophilia and the typical clinical manifestations. […] The treatment of CSS is similar to various other small-vessel vasculitis. High dose steroids are the mainstay of treatment and generally begin with a dose of 1mg/Kg/day (max 60mg/day) of prednisolone, maintained until symptoms are controlled and then slowly tapered (for one year).

• #2 Churg-Strauss syndrome (now called EGPA – Eosinophilic Granulomatosis with Polyangiitis)

  https://patient.info/doctor/churg-strauss-syndrome-pro

  EGPA is a rare disease. The estimated prevalence is 10.7 to 14 per million adults worldwide. […] The reported mean age of onset is 38-54 years, with a median of 40. However, it has been reported in extremes of age as well, from as low as 4 to as high as 74. […] There is no gender difference in incidence.

• #2 Churg-Strauss Syndrome or Eosinophilic Granulomatosis with Polyangiitis

  https://www.mdpi.com/2673-351X/1/1/24

  EGPA is a rare disease, with an estimated annual incidence of 0.5–4.2 cases/1,000,000 inhabitants, which has been found to be stable in recent years, with a prevalence of 11–14 cases/1,000,000 inhabitants. […] The knowledge and understanding of EGPA has evolved in recent years. Since anti-neutrophil cytoplasmic antibodies (ANCA) have been found in a proportion of patients, EGPA has subsequently been included in the spectrum of ANCA-associated vasculitis (AAV), along with GPA and microscopic polyangiitis (MPA). […] This review focuses on the areas of current evidence considering CSS classification, epidemiology, symptoms, diagnosis, prognosis, and treatment, especially from a respiratory approach, and attempts to identify the advances in the development and progression of the disease.

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

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

  The estimated prevalence of the disease is approximately 1 to 3 per million adults worldwide. […] About 40% of adults with EGPA have a positive ANCA, whereas 25% of children are seropositive for this. […] In contrast to certain small vessel vasculitides, such as immunoglobulin A (IgA) vasculitis and Kawasaki disease, with a preponderance in childhood, AAV is rare in children. […] Clinically, childhood EGPA has a higher incidence of cardiomyopathy and pneumonic infiltrates, with a lower occurrence of mononeuritis multiplex. […] The mean age of onset, as reported, ranges between 38 and 54, with a median of 40. […] Gender prevalences are similar between males and females.

• #2 AAIR :: Allergy, Asthma & Immunology Research

  https://e-aair.org/DOIx.php?id=10.4168/aair.2024.16.2.168

  Eosinophilic granulomatosis with polyangiitis (EGPA), formerly Churg-Strauss Syndrome, is a rare vasculitic disorder of small- to medium-size vessels. The incidence and prevalence of EGPA vary geographically and ethnically. This study investigated the incidence, prevalence, and mortality of EGPA in a nationwide population-based cohort in Korea. The mean annual standardized incidence between 2007 and 2018 was 1.2 (per 1,000,000 individuals). The incidence of EGPA has increased from 1.1 (per 1,000,000 individuals) in 2007 to 1.6 (per 1,000,000 individuals) in 2017. The standardized prevalence of EGPA has increased from 1.1(per 1,000,000 individuals) in 2007 to 11.2 (per 1,000,000 individuals) in 2018. The incidence and prevalence of EGPA were higher in women than in men. The overall mortality rate associated with EGPA was higher than that in the general population. Despite progress in understanding and treating EGPA, its epidemiology remains unclear. The prevalence of EGPA per 1,000,000 population was reported as 10.7 and 17.8 in two previous studies. The annual incidence of EGPA per 1,000,000 population ranges from 0.5 to 3.1. However, most epidemiologic studies were based on patients medical records from local hospitals or information obtained from hospital surveys. This study aimed to investigate the incidence, prevalence, and mortality rates of EGPA in a nationwide population-based cohort. The annual average standardized incidence from 2007 to 2018 and the standardized prevalence in 2018 per 1,000,000 individuals were 1.2 and 11.2, respectively, with a significant increase observed since 2014. Incidence and prevalence rates were higher in women than in men. The overall mortality rate of patients with EGPA was higher than that of the general population. Therefore, active investigation of EGPA for early detection is imperative when patients with asthma and blood eosinophilia present with symptoms affecting other organs to avoid delaying treatment, leading to an increased mortality risk.

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

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

  The estimated prevalence of Churg-Strauss disease (CSD), now known as eosinophilic granulomatosis with polyangiitis (EGPA), is 10.7 to 14 per million adults worldwide. There is no gender difference in the incidence of the disease. The age distribution is wide with reports of patients as young as 4 years and as old as 74 years; median age at presentation is 40 years old. […] The chief causes of mortality related to EGPA are severe asthma, cardiopulmonary failure, or gastrointestinal complications. In general, the long-term outcome of EGPA does not differ greatly from that of PAN. In addition to overall severity of EGPA, both myocardial disease and severe gastrointestinal disease were found by Guillevin et al to be independent predictors of poor prognosis. […] Across the years there has been a constant decrease in the mortality rate of patients who suffered from EGPA. Most (85%) of the index cases reported by Churg and Strauss in 1951 had died from their illness. The development of effective strategies for treatment in ensuing decades (particularly oral corticosteroids) has improved survival considerably, though the disease remains a serious one. To some extent, improved survival may relate to recognition of milder cases.

• #2 Eosinophilic granulomatosis with polyangiitis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/eosinophilic-granulomatosis-with-polyangiitis?embed_domain=fkgfeaby.ebecbve.dbz%255850%25588c3%25588eradiopaedia-icon-144.png&lang=us

  Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss syndrome is characterized by asthma, eosinophilia and small vessel necrotizing vasculitis and involves the lungs and other organs. […] The incidence typically peaks in middle age, 30-50 years of age with pediatric cases being rare. The annual incidence rate of around 0.24-1.78 per 100,000. There is no gender predilection.

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

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

  Churg-Strauss syndrome (CSS) is a rare small- and medium-sized-vessel vasculitis disease. […] Most CSS patients are adults in the third and fourth decades of life and there is a slight male predominance. […] Its prevalence, in the general population, ranges from 0.5 to 2.7 cases/million inhabitants. […] Skin lesions are important features of CSS, occur in about 55% of the cases and often occur in the third stage which is the most severe clinical stage. […] The diagnosis of CSS should based on the following 6 classification criteria, and 4 out of 6 criteria should present in the diagnosis of CSS: asthma, eosinophilia greater than 10%, paranasal sinusitis, unfixed pulmonary infiltration and histological proof of vasculitis and mono- or poly-neuropathy. […] Most of CSS patients are sensitive to systemic corticosteroids which are a first-line therapy for CSS.

• #2 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 […] EGPA is one of the rarest forms of vasculitis and primarily affects the small blood vessels. […] 11-14 Cases per Million Worldwide […] Number of US cases unknown […] Average age is 35-50, but can occur at any age […] Rare in children […] Ethnic prevalence unknown. […] EGPA is considered extremely rare. […] The cause of EGPA is not fully understood. […] Researchers believe an infection may set the inflammatory process in motion. […] Regular doctor visits and ongoing monitoring of lab and imaging tests are important in detecting relapses early. […] The majority of EGPA patients respond well to treatment. […] Because relapses are common with EGPA, follow-up medical care is essential.

• #2 Churg Strauss Syndrome – Vasculitis UK

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

  EGPA seems to affect men and women equally. Around 11-13 people per million are diagnosed with EGPA. […] Before treatment for EGPA was available, it was universally fatal. Half of patients diagnosed with EGPA died within 3 months of diagnosis. With modern treatment, this is much improved. We think that 8 in every 10 people diagnosed are still alive 5 years later.

• #2 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

  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. […] CSS prognosis is variable and depends on the initial extension of the disease and the organs affected. Five factors were described that are associated to a higher mortality among patients; which are: creatinine higher than 1.58mg/dL, proteinuria higher than 1 g/day, gastrointestinal involvement, central nervous system involvement and cardiomyopathy. […] Cutaneous manifestations of CSS are one type of clinical presentation for this condition. 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.

• #2 Peripheral Neuropathy as Initial Manifestation of Churg-Strauss Syndrome

  https://clinmedjournals.org/articles/iaim/international-archives-of-internal-medicine-iaim-3-021.php?jid=iaim

  EGPA have annual incidence rates of 0.5-3.7 per million in Europe. […] The relative rarity and non-specific presentation of the ANCA-associated vasculitides pose diagnostic challenges and often result in a significant diagnostic delay of more than 6 months in a third of patients. […] Neurological symptoms can be seen in 60-70% of patients with EGPA, secondary only to pulmonary manifestations in the overall frequency of organ involvement. […] An early recognition of this entity is the key in leading to timely treatment and positive outcome. […] The diagnosis of polyneuropathy is based on clinical and electrophysiologic studies, but precise histology, immunohistochemistry and morphometric studies of the peripheral nerve biopsy may be decisive to make diagnosis. […] In rare cases, vasculitis is confined to peripheral nerves without evidence of vasculitis in other organ systems.

• #3 Churg-Strauss syndrome (now called EGPA – Eosinophilic Granulomatosis with Polyangiitis)

  https://patient.info/doctor/churg-strauss-syndrome-pro

  EGPA is a rare disease. The estimated prevalence is 10.7 to 14 per million adults worldwide. […] The reported mean age of onset is 38-54 years, with a median of 40. However, it has been reported in extremes of age as well, from as low as 4 to as high as 74. […] There is no gender difference in incidence.

Zobacz więcej