Materiały źródłowe

• #1 Granulomatosis With Polyangiitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557827/

  Epidemiology: Among the 3 ANCA-associated vasculitides, GPA is the most common. The annual worldwide incidence of GPA is estimated to be 10 to 20 cases per million based on the geographical location. A higher incidence is noted in the colder regions. The prevalence of GPA in European and American populations is about 120 to 140 per million. A national study in the Netherlands on ANCA-associated vasculitis found that 167 patients (73%) were diagnosed with GPA, 54 (24%) with MPA, and 9 (4%) with EGPA. This distribution is similar to other European registries. […] GPA is more commonly reported in Whites, although it can be observed in all racial and ethnic groups. The onset of GPA occurs between 45 and 60 years, but a small proportion (3%-7%) affects children and adolescents. Children younger than 18 have a female-to-male predominance of about 2:1, while in adults, the ratio is 1:1.

• #2 Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis): Practice Essentials, Background, Etiology

  https://emedicine.medscape.com/article/332622-overview

  GPA is a rare disease with an as yet undetermined incidence. The prevalence of GPA in the United States is estimated to be 3 cases per 100,000 people. […] The incidence and prevalence of GPA in the United Kingdom is estimated at 10.2 cases and 250 cases per million population, respectively. […] GPA is more common in individuals of northern European descent (approximately 90%); it occurs less commonly in blacks. […] In European populations, GPA is slightly more common in men, with a male-to-female ratio of 1.5:1. […] The onset of GPA may occur at any age, although patients typically present at age 35-55 years. GPA is rare in childhood.

• #3 Epidemiology and Outcomes of Granulomatosis with Polyangiitis (GPA) in Pediatric and Working-age Adults Populations in the United States: Analysis of a Large National Claims Database

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6258356/

  The incidence and prevalence of Granulomatosis with Polyangiitis (GPA) in the U.S. is not well characterized. Owing to its rarity, outcomes data in pediatric-onset GPA are also lacking. The aims of this study were to describe the epidemiology and outcomes of GPA patients in the U.S. and compare outcomes between pediatric and working-age adult patients. […] The incidence rate of pediatric-onset GPA was 1.8 cases/million person-years compared to 12.8 cases/million person-years in working-age adults. […] This study represents the largest cohort of GPA reported to date. Pediatric GPA patients experienced more frequent hospitalizations and were more vulnerable to hematologic complications than non-elderly adult patients. […] Published epidemiology studies of GPA have primarily focused on Europe, the United Kingdom, and Japan. Estimates of the overall incidence range between 0.5-20 cases/million with a prevalence of 20-160 cases/million.

• #4 Granulomatosis With Polyangiitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557827/

  Epidemiology: Among the 3 ANCA-associated vasculitides, GPA is the most common. The annual worldwide incidence of GPA is estimated to be 10 to 20 cases per million based on the geographical location. A higher incidence is noted in the colder regions. The prevalence of GPA in European and American populations is about 120 to 140 per million. A national study in the Netherlands on ANCA-associated vasculitis found that 167 patients (73%) were diagnosed with GPA, 54 (24%) with MPA, and 9 (4%) with EGPA. This distribution is similar to other European registries. […] GPA is more commonly reported in Whites, although it can be observed in all racial and ethnic groups. The onset of GPA occurs between 45 and 60 years, but a small proportion (3%-7%) affects children and adolescents. Children younger than 18 have a female-to-male predominance of about 2:1, while in adults, the ratio is 1:1.

• #5 Epidemiology and Outcomes of Granulomatosis with Polyangiitis (GPA) in Pediatric and Working-age Adults Populations in the United States: Analysis of a Large National Claims Database

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6258356/

  Given the paucity of epidemiology and outcome data of GPA in both adults and children, we performed a study using a large insurance claims dataset from the years 2006-2014 to identify and characterize both pediatric and adult GPA patients in the United States. […] The prevalence of GPA was 3.4 cases per million person-years in children and 30.5 cases per million person-years in adults less than 65 years of age. […] Our study demonstrated a slight female predominance in both pediatric and adult GPA cohorts, confirming the female predominance of pediatric GPA which has been observed in nearly all previous studies. […] Our study found an incidence rate of 12.8 per million person-years in the U.S. which is comparable with a prior smaller UK report which found an incidence rate of 14.0 per million person-years.

• #6 Granulomatosis With Polyangiitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557827/

  Epidemiology: Among the 3 ANCA-associated vasculitides, GPA is the most common. The annual worldwide incidence of GPA is estimated to be 10 to 20 cases per million based on the geographical location. A higher incidence is noted in the colder regions. The prevalence of GPA in European and American populations is about 120 to 140 per million. A national study in the Netherlands on ANCA-associated vasculitis found that 167 patients (73%) were diagnosed with GPA, 54 (24%) with MPA, and 9 (4%) with EGPA. This distribution is similar to other European registries. […] GPA is more commonly reported in Whites, although it can be observed in all racial and ethnic groups. The onset of GPA occurs between 45 and 60 years, but a small proportion (3%-7%) affects children and adolescents. Children younger than 18 have a female-to-male predominance of about 2:1, while in adults, the ratio is 1:1.

• #7 Granulomatosis with Polyangiitis (GPA) – EyeWiki

  https://eyewiki.org/Granulomatosis_with_Polyangiitis_(GPA)

  Granulomatosis with polyangiitis (GPA) is an autoimmune disorder characterized by granulomatous necrotizing vasculitis that typically affects small and/or medium sized blood vessels in the orbit, respiratory tract (sinuses, nose, throat, lungs), and kidneys. […] Of the three ANCA-associated vasculitides, GPA is the most common. The annual incidence is about 810 cases per million and the prevalence of GPA is estimated to be 3 cases per 100,000 people. […] These numbers vary by continent but GPA is typically more common in colder climates. […] Patients are usually whited and in their 40s-50s but GPA can occur in any age or race. […] Ocular disease, one of the earliest possible disease manifestations, is seen in at least 15% of cases of GPA, and is much less frequently associated with MPA or CSS.

• #8 Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis): Practice Essentials, Background, Etiology

  https://emedicine.medscape.com/article/332622-overview

  GPA is a rare disease with an as yet undetermined incidence. The prevalence of GPA in the United States is estimated to be 3 cases per 100,000 people. […] The incidence and prevalence of GPA in the United Kingdom is estimated at 10.2 cases and 250 cases per million population, respectively. […] GPA is more common in individuals of northern European descent (approximately 90%); it occurs less commonly in blacks. […] In European populations, GPA is slightly more common in men, with a male-to-female ratio of 1.5:1. […] The onset of GPA may occur at any age, although patients typically present at age 35-55 years. GPA is rare in childhood.

• #9 Granulomatosis With Polyangiitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557827/

  Epidemiology: Among the 3 ANCA-associated vasculitides, GPA is the most common. The annual worldwide incidence of GPA is estimated to be 10 to 20 cases per million based on the geographical location. A higher incidence is noted in the colder regions. The prevalence of GPA in European and American populations is about 120 to 140 per million. A national study in the Netherlands on ANCA-associated vasculitis found that 167 patients (73%) were diagnosed with GPA, 54 (24%) with MPA, and 9 (4%) with EGPA. This distribution is similar to other European registries. […] GPA is more commonly reported in Whites, although it can be observed in all racial and ethnic groups. The onset of GPA occurs between 45 and 60 years, but a small proportion (3%-7%) affects children and adolescents. Children younger than 18 have a female-to-male predominance of about 2:1, while in adults, the ratio is 1:1.

• #10 Granulomatosis with polyangiitis – Wikipedia

  https://en.wikipedia.org/wiki/Granulomatosis_with_polyangiitis

  The number of new cases of GPA each year is estimated to be between 2.1 and 14.4 new cases per million people in Europe. […] GPA is rare in Japanese and African-American populations but occurs more often in people of Northern European descent. […] GPA is estimated to affect three cases per 100,000 people in the United States and affects men and women equally. […] The incidence is 1020 cases per million per year. […] It is exceedingly rare in Japan and among African Americans.

• #11 Granulomatosis With Polyangiitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557827/

  Epidemiology: Among the 3 ANCA-associated vasculitides, GPA is the most common. The annual worldwide incidence of GPA is estimated to be 10 to 20 cases per million based on the geographical location. A higher incidence is noted in the colder regions. The prevalence of GPA in European and American populations is about 120 to 140 per million. A national study in the Netherlands on ANCA-associated vasculitis found that 167 patients (73%) were diagnosed with GPA, 54 (24%) with MPA, and 9 (4%) with EGPA. This distribution is similar to other European registries. […] GPA is more commonly reported in Whites, although it can be observed in all racial and ethnic groups. The onset of GPA occurs between 45 and 60 years, but a small proportion (3%-7%) affects children and adolescents. Children younger than 18 have a female-to-male predominance of about 2:1, while in adults, the ratio is 1:1.

• #12 Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis): Practice Essentials, Background, Etiology

  https://emedicine.medscape.com/article/332622-overview

  GPA is a rare disease with an as yet undetermined incidence. The prevalence of GPA in the United States is estimated to be 3 cases per 100,000 people. […] The incidence and prevalence of GPA in the United Kingdom is estimated at 10.2 cases and 250 cases per million population, respectively. […] GPA is more common in individuals of northern European descent (approximately 90%); it occurs less commonly in blacks. […] In European populations, GPA is slightly more common in men, with a male-to-female ratio of 1.5:1. […] The onset of GPA may occur at any age, although patients typically present at age 35-55 years. GPA is rare in childhood.

• #13 Granulomatosis with polyangiitis – Wikipedia

  https://en.wikipedia.org/wiki/Granulomatosis_with_polyangiitis

  The number of new cases of GPA each year is estimated to be between 2.1 and 14.4 new cases per million people in Europe. […] GPA is rare in Japanese and African-American populations but occurs more often in people of Northern European descent. […] GPA is estimated to affect three cases per 100,000 people in the United States and affects men and women equally. […] The incidence is 1020 cases per million per year. […] It is exceedingly rare in Japan and among African Americans.

• #14

  https://reu.termedia.pl/Granulomatosis-with-polyangiitis-in-Poland-epidemiological-study,57235,0,2.html

  Granulomatosis with polyangiitis (GPA) is a disease of unknown aetiology, with a variable course, and with the possibility of exacerbations associated with life-threatening organ damage. Data on the incidence of GPA in Poland are limited. Epidemiological analysis can provide a lot of information that contributes to more accurate knowledge about GPA. The objective of this study was to conduct an analysis of GPA incidence in Poland with comparison of results to selected European countries. An additional analysis was performed according to the distribution of gender, age and hospitalization structure in GPA patients. […] The average annual incidence of GPA in Poland was estimated to be 4.9/million in the general population, 5.8/million in the adult population and 1/million in the population of patients below 18 years of age. The annual incidence in Poland was comparable to incidence data from other European countries. […] In this study GPA incidence in Poland was estimated. Analyses of the structure of hospitalizations suggested that symptoms from the respiratory tract, kidneys and joints could be leading symptoms in the early stage of GPA development.

• #15 Epidemiology and Treatment Outcome of ANCA-associated Vasculitis in South Korea: A Nationwide, Population-based Cohort Study – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/epidemiology-and-treatment-outcome-of-anca-associated-vasculitis-in-south-korea-a-nationwide-population-based-cohort-study/

  Results: A total of 546 and 795 patients with GPA and MPA, respectively, were identified. The incidence rates of both diseases increased with age, and the peak incidence rate was observed in patients aged 70 years. GPA and MPA showed different trends in incidence rates over time. In 2010, the annual incidence rates (per million) of GPA and MPA were 1.03 and 0.83, respectively. The incidence of MPA has continuously increased over time and increased to 3.26 in 2018. In contrast, the annual incidence of GPA did not significantly change during the observation period and was 1.33 in 2018. […] During the observation period, 90 (28.3%) and 187 (38.6%) patients in the GPA and MPA groups, respectively, died. Although all-cause mortality rates in the GPA and MPA group were significantly higher compared to that in each comparator group (adjusted HR 5.15 [3.21 to 8.38] and 8.62 [6.52 to 11.4], respectively), it was even higher in patients with MPA (adjusted HR 1.69 [1.31 to 2.18]). Patients in the GPA and MPA groups also showed a significantly higher risk of renal replacement therapy and ICU admission than those in the comparator population. In both groups, mortality and morbidity outcomes occurred mainly within the first year from the diagnosis of underlying vasculitis. […] Conclusion: In South Korea, the incidence of MPA has increased over time. Although both GPA and MPA had high rates of mortality and morbidity, MPA has a poorer prognosis than GPA.

• #16 Granulomatosis With Polyangiitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557827/

  Epidemiology: Among the 3 ANCA-associated vasculitides, GPA is the most common. The annual worldwide incidence of GPA is estimated to be 10 to 20 cases per million based on the geographical location. A higher incidence is noted in the colder regions. The prevalence of GPA in European and American populations is about 120 to 140 per million. A national study in the Netherlands on ANCA-associated vasculitis found that 167 patients (73%) were diagnosed with GPA, 54 (24%) with MPA, and 9 (4%) with EGPA. This distribution is similar to other European registries. […] GPA is more commonly reported in Whites, although it can be observed in all racial and ethnic groups. The onset of GPA occurs between 45 and 60 years, but a small proportion (3%-7%) affects children and adolescents. Children younger than 18 have a female-to-male predominance of about 2:1, while in adults, the ratio is 1:1.

• #17 Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis): Practice Essentials, Background, Etiology

  https://emedicine.medscape.com/article/332622-overview

  GPA is a rare disease with an as yet undetermined incidence. The prevalence of GPA in the United States is estimated to be 3 cases per 100,000 people. […] The incidence and prevalence of GPA in the United Kingdom is estimated at 10.2 cases and 250 cases per million population, respectively. […] GPA is more common in individuals of northern European descent (approximately 90%); it occurs less commonly in blacks. […] In European populations, GPA is slightly more common in men, with a male-to-female ratio of 1.5:1. […] The onset of GPA may occur at any age, although patients typically present at age 35-55 years. GPA is rare in childhood.

• #18 Granulomatosis with Polyangiitis (GPA) – Musculoskeletal and Connective Tissue Disorders – Merck Manual Professional Edition

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

  Granulomatosis with polyangiitis is characterized by necrotizing granulomatous inflammation, small- and medium-sized vessel vasculitis, and focal necrotizing glomerulonephritis, often with crescent formation. […] The incidence of granulomatosis with polyangiitis (GPA) varies depending on the population being studied. For example, a large cohort study in the United Kingdom reported an incidence of 11.8/million person-years. It is most common among people of European ancestry but can occur in all ethnic groups and at any age. Mean age at onset is 40 years. […] Granulomatosis with polyangiitis should be suspected in patients with chronic, unexplained respiratory symptoms and signs, particularly if manifestations in other organ systems, especially the kidneys, also suggest the disorder. […] Treatment of granulomatosis with polyangiitis depends on the severity of disease. A multidisciplinary approach is required for multiorgan disease, often including a rheumatologist, otorhinolaryngologist, pulmonologist, and nephrologist.

• #19 Epidemiology and Outcomes of Granulomatosis with Polyangiitis (GPA) in Pediatric and Working-age Adults Populations in the United States: Analysis of a Large National Claims Database

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6258356/

  The incidence and prevalence of Granulomatosis with Polyangiitis (GPA) in the U.S. is not well characterized. Owing to its rarity, outcomes data in pediatric-onset GPA are also lacking. The aims of this study were to describe the epidemiology and outcomes of GPA patients in the U.S. and compare outcomes between pediatric and working-age adult patients. […] The incidence rate of pediatric-onset GPA was 1.8 cases/million person-years compared to 12.8 cases/million person-years in working-age adults. […] This study represents the largest cohort of GPA reported to date. Pediatric GPA patients experienced more frequent hospitalizations and were more vulnerable to hematologic complications than non-elderly adult patients. […] Published epidemiology studies of GPA have primarily focused on Europe, the United Kingdom, and Japan. Estimates of the overall incidence range between 0.5-20 cases/million with a prevalence of 20-160 cases/million.

• #20 Epidemiology and Outcomes of Granulomatosis with Polyangiitis (GPA) in Pediatric and Working-age Adults Populations in the United States: Analysis of a Large National Claims Database

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6258356/

  To our knowledge, our study represents the first large epidemiologic study of pediatric GPA, encompassing 214 pediatric GPA patients. We found an incidence rate of 1.8 per million person-years among children. […] The overall rate of ESRD appeared higher in pediatric patients compared to adults 65 years of age but this was not statistically significant. […] In summary, this large cohort of pediatric and working-age adult GPA patients provides novel information on incidence, prevalence, and outcomes of GPA in the United States.

• #21 Granulomatosis with Polyangiitis Rates & Outcomes – The Rheumatologist

  https://www.the-rheumatologist.org/article/granulomatosis-with-polyangiitis-rates-outcomes/

  A recent study in Arthritis Rheumatology highlights new information about the epidemiology and disease course of the vasculitic disease granulomatosis with polyangiitis (GPA, formerly known as Wegeners disease). GPA is a rare disease thats generally specific to the lungs, kidneys and the upper airways. The study provides key new data about the incidence and prevalence of the disease in the U.S. It also sheds light on disease course and outcomes. […] Information about GPA has been particularly limited for pediatric populations. Due to its relative rarity in children, much less has been known about the disease in this group, with studies limited to small cohorts. […] Overall, the researchers identified 5,566 cases of GPA. Of these, 3.8% (214) were pediatric cases. The incidence rate was 12.8 cases per million person-years in working-age adults (i.e., less than age 65), with a lower rate of 1.8 cases per million person-years for children.

• #22 Granulomatosis With Polyangiitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557827/

  Epidemiology: Among the 3 ANCA-associated vasculitides, GPA is the most common. The annual worldwide incidence of GPA is estimated to be 10 to 20 cases per million based on the geographical location. A higher incidence is noted in the colder regions. The prevalence of GPA in European and American populations is about 120 to 140 per million. A national study in the Netherlands on ANCA-associated vasculitis found that 167 patients (73%) were diagnosed with GPA, 54 (24%) with MPA, and 9 (4%) with EGPA. This distribution is similar to other European registries. […] GPA is more commonly reported in Whites, although it can be observed in all racial and ethnic groups. The onset of GPA occurs between 45 and 60 years, but a small proportion (3%-7%) affects children and adolescents. Children younger than 18 have a female-to-male predominance of about 2:1, while in adults, the ratio is 1:1.

• #23 Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis): Practice Essentials, Background, Etiology

  https://emedicine.medscape.com/article/332622-overview

  GPA is a rare disease with an as yet undetermined incidence. The prevalence of GPA in the United States is estimated to be 3 cases per 100,000 people. […] The incidence and prevalence of GPA in the United Kingdom is estimated at 10.2 cases and 250 cases per million population, respectively. […] GPA is more common in individuals of northern European descent (approximately 90%); it occurs less commonly in blacks. […] In European populations, GPA is slightly more common in men, with a male-to-female ratio of 1.5:1. […] The onset of GPA may occur at any age, although patients typically present at age 35-55 years. GPA is rare in childhood.

• #24 Epidemiology and Outcomes of Granulomatosis with Polyangiitis (GPA) in Pediatric and Working-age Adults Populations in the United States: Analysis of a Large National Claims Database

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6258356/

  Given the paucity of epidemiology and outcome data of GPA in both adults and children, we performed a study using a large insurance claims dataset from the years 2006-2014 to identify and characterize both pediatric and adult GPA patients in the United States. […] The prevalence of GPA was 3.4 cases per million person-years in children and 30.5 cases per million person-years in adults less than 65 years of age. […] Our study demonstrated a slight female predominance in both pediatric and adult GPA cohorts, confirming the female predominance of pediatric GPA which has been observed in nearly all previous studies. […] Our study found an incidence rate of 12.8 per million person-years in the U.S. which is comparable with a prior smaller UK report which found an incidence rate of 14.0 per million person-years.

• #25 Pathology Outlines – Granulomatosis with polyangiitis (GPA)

  https://www.pathologyoutlines.com/topic/lungnontumorgranulomatosiswithpolyangiitis.html

  Granulomatosis with polyangiitis (GPA) is a systemic autoimmune vasculitis syndrome commonly involving the lower respiratory tract, the upper respiratory tract and the kidney. […] Epidemiology: Incidence: M = F (slight male predominance). Mean age: 50 years (Int J Immunopathol Pharmacol 2016;29:151). Rare in children and young adults. Caucasian population of European descent is most commonly affected. […] Risk factors: Infectious, environmental and drug induced triggers. First degree relatives with GPA. HLA-DPB1*0401 variant as genetic risk factor (Ann Rheum Dis 2011;70:707). Thyroid disease: Graves disease and Hashimoto thyroiditis (Arthritis Rheum 2003;48:2299).

• #26 Granulomatosis with polyangiitis (Wegener’s granulomatosis)

  https://patient.info/doctor/granulomatosis-with-polyangiitis-wegeners-granulomatosis-pro

  The higher incidence in winter suggests an infective aetiology but the data are inconclusive. GPA has been linked to parvovirus and to chronic nasal carriage of Staphylococcus aureus. […] The concept that successful management not only relies on the suppression of inflammation but also on minimising chronic morbidity (’damage’) is gaining ground. This involves surveillance for associated diseases such as malignancy, venous thromboembolic events and cardiovascular disease as well as minimising the risk of adverse drug effects such as renal toxicity.

• #27 Pathology Outlines – Granulomatosis with polyangiitis (GPA)

  https://www.pathologyoutlines.com/topic/lungnontumorgranulomatosiswithpolyangiitis.html

  Granulomatosis with polyangiitis (GPA) is a systemic autoimmune vasculitis syndrome commonly involving the lower respiratory tract, the upper respiratory tract and the kidney. […] Epidemiology: Incidence: M = F (slight male predominance). Mean age: 50 years (Int J Immunopathol Pharmacol 2016;29:151). Rare in children and young adults. Caucasian population of European descent is most commonly affected. […] Risk factors: Infectious, environmental and drug induced triggers. First degree relatives with GPA. HLA-DPB1*0401 variant as genetic risk factor (Ann Rheum Dis 2011;70:707). Thyroid disease: Graves disease and Hashimoto thyroiditis (Arthritis Rheum 2003;48:2299).

• #28 Granulomatosis with polyangiitis (Wegener’s granulomatosis)

  https://patient.info/doctor/granulomatosis-with-polyangiitis-wegeners-granulomatosis-pro

  The higher incidence in winter suggests an infective aetiology but the data are inconclusive. GPA has been linked to parvovirus and to chronic nasal carriage of Staphylococcus aureus. […] The concept that successful management not only relies on the suppression of inflammation but also on minimising chronic morbidity (’damage’) is gaining ground. This involves surveillance for associated diseases such as malignancy, venous thromboembolic events and cardiovascular disease as well as minimising the risk of adverse drug effects such as renal toxicity.

• #29 Granulomatosis with polyangiitis (Wegener’s granulomatosis)

  https://patient.info/doctor/granulomatosis-with-polyangiitis-wegeners-granulomatosis-pro

  Granulomatosis with polyangiitis epidemiology […] A study using information from the UK General Practice Research Database reported an overall annual incidence of 8.4/million. […] One study looking at GPA as a cause of renal vasculitis showed that the annual incidence of such cases in the UK was 5.8/million. The incidence was found to be lower in Japan. […] The male:female ratio is approximately 1.2:1. […] The condition can occur at any age but peaks between the ages of 35-55. One American study found that the incidence in children was increasing. […] Another study found that first-degree relatives had a moderately increased risk of developing any autoimmune/inflammatory disease, including specific associations with, for example, multiple sclerosis, Sjgren’s syndrome and seropositive rheumatoid arthritis.

• #30 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.

• #31 Orphanet: Granulomatosis with polyangiitis

  https://www.orpha.net/en/disease/detail/900

  The prevalence is estimated between 1/6,400 – 42,000 worldwide with annual incidence between 1/84,000-475,000. There is geographic and/or ethnic variation, with a higher incidence in colder regions and among Caucasians. Childhood-onset disease is characterized by female predominance, and adult-onset by a slight male predominance. […] Diagnosis relies on clinical findings, imaging studies and biochemical testing, and detection of ANCAs in the serum, principally PR3-ANCAs. Histological analysis of biopsy specimens from affected organs confirms the diagnosis, showing necrotizing vasculitis and granulomatous inflammation.

• #32 Granulomatosis with Polyangiitis initially misdiagnosed as lung cancer | Pulmonology

  https://journalpulmonology.org/en-granulomatosis-with-polyangiitis-initially-misdiagnosed-articulo-S2173511512000504

  cANCA reacting to PR3 is very (but not totally) specific of GPA, it is found in approximately 90% of patients with active generalized GPA and 60% of active limited GPA. […] A definite histological diagnosis of GPA requires coexistence of vasculitis, granuloma and necrosis although these features are only found in a minority of specimens. Only 1 or 2 of these features may be found, rendering a suggestive biopsy, in which the diagnosis is then supported by clinical criteria. […] Lung nodules are the most common pulmonary manifestation of Wegener’s granulomatosis and occur in approximately 40-70% of patients. Nodules are usually multiple and bilateral and occur without a zonal predilection. […] Solitary or multifocal involvement of the lung by GPA without evidence of extrapulmonary disease is sometimes a difficult diagnosis due to its histopathological variability and possible misinterpretation as lung cancer. […] The recurrence of the generalized form of GPA, although initially responsive to preconized therapy, was complicated by several infectious events and progressive respiratory failure that led to the death of the patient.

• #33 Granulomatosis with Polyangiitis (GPA)—A Multidisciplinary Approach of a Case Report

  https://www.mdpi.com/1648-9144/58/12/1837

  Laboratory tests show elevated levels of anti-neutrophil cytoplasmic antibodies (ANCA) with a cytoplasmic staining pattern directed against proteinase 3 (PR3). Being related to disease activity, ANCAs have been identified as risk factors of GPA relapse and are currently used in the long-term follow-up process. […] Granulomatosis with polyangiitis is a rare and fatal disease without treatment. Early diagnosis and treatment are key to controlling its progression. Necrotizing granulomatous inflammation and vasculitis on a biopsy, along with the presence of ANCAs, provide support for the diagnosis of the disease. Various manifestations occupying a wide distribution may be found throughout disease progression. Regular ophthalmic examination is important since a multitude of manifestations can be present.

• #34 Granulomatosis with Polyangiitis (GPA) – EyeWiki

  https://eyewiki.org/Granulomatosis_with_Polyangiitis_(GPA)

  The upper respiratory tract (sinuses, nose, ears, and trachea) is the most often involved system, with up to 85% of patients having disease, and 81% of patients having ENT findings as their initial symptoms. […] Renal involvement occurs in 75% of patients with the disease and can present with symptoms of glomerulonephritis. […] The American College of Rheumatology (ACR) has designated a diagnostic criteria for GPA. […] The presence of two out of four of these criteria is 88.2% and 92% sensitive and specific, respectively. […] The annual incidence is about 810 cases per million and the prevalence of GPA is estimated to be 3 cases per 100,000 people.

• #35 Granulomatosis with Polyangiitis (GPA) – EyeWiki

  https://eyewiki.org/Granulomatosis_with_Polyangiitis_(GPA)

  The upper respiratory tract (sinuses, nose, ears, and trachea) is the most often involved system, with up to 85% of patients having disease, and 81% of patients having ENT findings as their initial symptoms. […] Renal involvement occurs in 75% of patients with the disease and can present with symptoms of glomerulonephritis. […] The American College of Rheumatology (ACR) has designated a diagnostic criteria for GPA. […] The presence of two out of four of these criteria is 88.2% and 92% sensitive and specific, respectively. […] The annual incidence is about 810 cases per million and the prevalence of GPA is estimated to be 3 cases per 100,000 people.

• #36

  https://link.springer.com/article/10.1007/s42399-019-00083-4

  GPA is a type of ANCA-associated vasculitis. […] Various factors may have an impact on the etiology of GPA which is treated as an autoimmune disease. […] GPA is known as a disease affecting the upper and lower respiratory tracts and kidneys and associated with otorhinolaryngological and renal manifestations. […] The mortality of untreated GPA is high, so the issue of effective treatment is vital. […] The frequency of pericarditis may concern even 6% of patients with GPA and may occur secondary to myocardial infarction or uremia in the course of kidney involvement. […] The definition of GPA includes necrotizing vasculitis concerning small-sized and medium-sized vessels but there are also known cases involving large vessels. […] The literature shows an approximate 1026% prevalence of gastrointestinal tract involvement. […] Articular or muscular abnormalities concern 4.767% with the main complaint being arthralgia (generally polyarthalgia) of the knees, hips, wrists, or ankles. […] The prevalence of ocular changes occurs in 3058% of cases.

• #37 Granulomatosis with Polyangiitis (GPA) – EyeWiki

  https://eyewiki.org/Granulomatosis_with_Polyangiitis_(GPA)

  The upper respiratory tract (sinuses, nose, ears, and trachea) is the most often involved system, with up to 85% of patients having disease, and 81% of patients having ENT findings as their initial symptoms. […] Renal involvement occurs in 75% of patients with the disease and can present with symptoms of glomerulonephritis. […] The American College of Rheumatology (ACR) has designated a diagnostic criteria for GPA. […] The presence of two out of four of these criteria is 88.2% and 92% sensitive and specific, respectively. […] The annual incidence is about 810 cases per million and the prevalence of GPA is estimated to be 3 cases per 100,000 people.

• #38

  https://link.springer.com/article/10.1007/s42399-019-00083-4

  GPA is a type of ANCA-associated vasculitis. […] Various factors may have an impact on the etiology of GPA which is treated as an autoimmune disease. […] GPA is known as a disease affecting the upper and lower respiratory tracts and kidneys and associated with otorhinolaryngological and renal manifestations. […] The mortality of untreated GPA is high, so the issue of effective treatment is vital. […] The frequency of pericarditis may concern even 6% of patients with GPA and may occur secondary to myocardial infarction or uremia in the course of kidney involvement. […] The definition of GPA includes necrotizing vasculitis concerning small-sized and medium-sized vessels but there are also known cases involving large vessels. […] The literature shows an approximate 1026% prevalence of gastrointestinal tract involvement. […] Articular or muscular abnormalities concern 4.767% with the main complaint being arthralgia (generally polyarthalgia) of the knees, hips, wrists, or ankles. […] The prevalence of ocular changes occurs in 3058% of cases.

• #39 The eye is a common site of granulomatosis with polyangiitis. A collaborative study | BMC Ophthalmology | Full Text

  https://bmcophthalmol.biomedcentral.com/articles/10.1186/s12886-022-02743-x

  Ocular manifestations have been recorded with frequencies ranging from 13% to 60% and are either the sole presenting feature (~15% of patients) or, as is more often the case, appear at variable time during the course of the disease. […] In our cohort of 63 patients, ocular manifestations were detected in 22 patients (34.9%) and similar percentages have been reported in the USA (30.1%) and France (38.6%). However, a study in Spain determined a remarkably lower incidence (13.3%) and studies in Russia (50%) and the USA (58%) a significantly higher incidence of the disease. […] Overall, no correlation could be established between the type of ocular lesion and the location and extent of systemic manifestations. However, 15 of the 22 patients (68.2%) with ocular involvement had combined ocular and sino-nasal symptoms, a strikingly high frequency suggesting that ocular involvement is often the consequence of contiguous disease spread from the paranasal sinuses. […] Based on the data obtained in this cohort, we recommend an ophthalmological examination for all GPA patients to allow the early recognition of ocular injury and thus the prevention of the often severe consequences of a late diagnosis.

• #40 Epidemiology and Outcomes of Granulomatosis with Polyangiitis (GPA) in Pediatric and Working-age Adults Populations in the United States: Analysis of a Large National Claims Database

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6258356/

  The incidence and prevalence of Granulomatosis with Polyangiitis (GPA) in the U.S. is not well characterized. Owing to its rarity, outcomes data in pediatric-onset GPA are also lacking. The aims of this study were to describe the epidemiology and outcomes of GPA patients in the U.S. and compare outcomes between pediatric and working-age adult patients. […] The incidence rate of pediatric-onset GPA was 1.8 cases/million person-years compared to 12.8 cases/million person-years in working-age adults. […] This study represents the largest cohort of GPA reported to date. Pediatric GPA patients experienced more frequent hospitalizations and were more vulnerable to hematologic complications than non-elderly adult patients. […] Published epidemiology studies of GPA have primarily focused on Europe, the United Kingdom, and Japan. Estimates of the overall incidence range between 0.5-20 cases/million with a prevalence of 20-160 cases/million.

• #41 Granulomatosis with Polyangiitis Rates & Outcomes – The Rheumatologist

  https://www.the-rheumatologist.org/article/granulomatosis-with-polyangiitis-rates-outcomes/

  The adult rate is comparable to that of a smaller report from the United Kingdom that reported a rate of 14.0 per million person-years. The pediatric rate was also in line with two other much smaller studies that found incidence rates of 2.8 and 0.9 cases per million person-years. […] The researchers also studied complication rates. Both children and working-age adults had high rates of hospitalizations and severe infections. We found that the most common complications were being hospitalized, having a severe infection and having hematologic abnormalities such as cytopenias, says Dr. White. We found that hospitalizations were a little more common in children. Of the severe infections, they were mostly of the respiratory tractpneumonias. The hematologic complications were more common in children than adults as well. In fact, children had two to three times higher rates of leukopenia, neutropenia and hypogammaglobulinemia. Pulmonary infections occurred at a rate of approximately 50 infections per 1,000 person-years in both groups.

• #42 Granulomatosis with polyangiitis | Radiology Reference Article | Radiopaedia.org

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

  Granulomatosis with polyangiitis (GPA), previously known as Wegener granulomatosis, is a multisystem necrotizing non-caseating granulomatous c-ANCA positive vasculitis affecting small to medium-sized arteries, capillaries, and veins, with a predilection for the respiratory system and kidneys. […] There is a slight male predilection, and onset is typically at approximately 50 years of age. […] Without treatment, granulomatosis with polyangiitis is rapidly progressive with death usually occurring within a year of diagnosis, with only 10% of patients surviving 2 years. Appropriate medical therapy has dramatically increased long-term survival.

• #43 Granulomatosis with polyangiitis: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/000135.htm

  Granulomatosis with polyangiitis (GPA) is a rare disorder in which blood vessels become inflamed. This leads to damage in major organs of the body. It was formerly known as Wegener’s granulomatosis. […] GPA is most common in middle-aged adults of northern European descent. It is rare in children. […] Without treatment, people with severe forms of this disease can die within a few months. […] With treatment, the outlook for most patients is good. Most people who receive corticosteroids and other medicines that slow the immune response get much better. Most people with GPA are treated with ongoing medicines to prevent relapse for at least 12 to 24 months. […] Complications most often occur when the disease is not treated. People with GPA develop tissue damage in the lungs, airways, and the kidneys. Kidney involvement may result in blood in the urine and kidney failure. Kidney disease can quickly get worse. Kidney function may not improve, even when the condition is controlled by medicines. […] If untreated, kidney failure and possibly death occur in most cases.

• #44 Granulomatosis with polyangiitis: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/000135.htm

  Granulomatosis with polyangiitis (GPA) is a rare disorder in which blood vessels become inflamed. This leads to damage in major organs of the body. It was formerly known as Wegener’s granulomatosis. […] GPA is most common in middle-aged adults of northern European descent. It is rare in children. […] Without treatment, people with severe forms of this disease can die within a few months. […] With treatment, the outlook for most patients is good. Most people who receive corticosteroids and other medicines that slow the immune response get much better. Most people with GPA are treated with ongoing medicines to prevent relapse for at least 12 to 24 months. […] Complications most often occur when the disease is not treated. People with GPA develop tissue damage in the lungs, airways, and the kidneys. Kidney involvement may result in blood in the urine and kidney failure. Kidney disease can quickly get worse. Kidney function may not improve, even when the condition is controlled by medicines. […] If untreated, kidney failure and possibly death occur in most cases.

• #45 Predictors of relapse in granulomatosis with polyangiitis: a multi-center study | Egyptian Rheumatology and Rehabilitation | Full Text

  https://erar.springeropen.com/articles/10.1186/s43166-022-00160-y

  Granulomatosis with polyangiitis (GPA) is a rare systemic vasculitis. Its severity ranges from indolent disease to fulminant that may cause death. With treatment, remission is seen in more than 80% of cases, although relapse is still common. There have been studies showing that there may be factors to predict relapse in GPA. The annual incidence of the disease is estimated as 421 cases per million. The peak incidence is seen in 47th decades of life. Men and women are equally affected. Although the etiology of GPA remains unknown, studies have shown that the roles of genetic, immunologic, environmental factors, and microbial pathogens have been influential. GPA is usually associated with antibodies against neutrophils (antineutrophilic cytoplasmic antibodies (ANCA)) and so the disease is a component of a spectrum of disorders entitled the ANCA-associated vasculitides (AAVs). Studies show that using immunosuppressants combined with corticosteroids fundamentally improves the prognosis of GPA. With treatment, remission is seen in more than 80% of cases, although relapse is still common (happens in more than 50% of cases). There have been studies showing that there may be factors to predict relapse in GPA. For example, some studies showed that ear, nose, and throat (ENT) involvement is associated with more relapses. Presence of PR3-ANCA and cardiac involvement at the time of diagnosis, are other factors associated with increased risk of relapsing. A number of studies have shown that relapses are much more common in patients with positive PR3-ANCA than in those with positive MPO-ANCA. Based on these relapses and predicting factors, the decision to start treatment and/or to monitor the patients more closely is made. Therefore, predicting the relapse of GPA can be effective in controlling the disease. Among clinical and laboratory features we studied, positive PR3-ANCA, nose manifestations, mucosal membranes involvement, and GI complications were independent predictors of relapse in patients with GPA.

• #46 Score to assess the probability of relapse in granulomatosis with polyangiitis and microscopic polyangiitis | RMD Open

  https://rmdopen.bmj.com/content/9/1/e002953

  A higher risk of relapse was independently associated with PR3-ANCA (HR=1.81 (95% CI 1.28 to 2.57); p0.001), age 75 years (HR=1.89 (95% CI 1.15 to 3.13); p=0.012) and eGFR (CKD-EPI) 30mL/min/1.73m (HR=1.67 (95% CI 1.18 to 2.33); p=0.004) at AAV diagnosis. […] This study provides a simple score, entitled FRS, which can be calculated at the time of diagnosis of GPA or MPA to predict the risk of relapse during follow-up and which may ultimately allow clinicians to tailor the duration of maintenance therapy for these vasculitides. […] The FRS is not applicable to EGPA and PAN patients, who were not included in this study. In PAN, the risk of relapse is lower. […] Our study has many strengths: the reliability of the data (data from prospective therapeutic trials), the use of a Fine-Gray model to distinguish between two competitive risks (death and relapse), the analysis stratified on the induction and maintenance treatment (which influences the progression of the disease), and its validation in an independent cohort, which allowed us to show that the FRS reliably predicts the risk of relapse and that its level of performance increases with time.

• #47 Granulomatosis with Polyangiitis (Wegener’s)

  https://rheumatology.org/patients/granulomatosis-with-polyangiitis-wegeners

  Granulomatosis with polyangiitis (GPA), formerly called Wegeners granulomatosis, is a rare blood vessel disease. GPA is a rare condition, affecting only 3 out of every 100,000 people, typically aged 40 to 65 and equally affecting women and men. […] People with GPA need regular check-ups, as disease relapses are common. It can also have serious complications that are even life-threatening if not treated promptly. Patients need regular laboratory tests, imaging scans and clinical visits with a rheumatology provider. Regular rheumatology visits and testing should spot any problems early so that prompt, effective treatment can be started.

• #48 Granulomatosis with polyangiitis (Wegener’s granulomatosis)

  https://patient.info/doctor/granulomatosis-with-polyangiitis-wegeners-granulomatosis-pro

  The higher incidence in winter suggests an infective aetiology but the data are inconclusive. GPA has been linked to parvovirus and to chronic nasal carriage of Staphylococcus aureus. […] The concept that successful management not only relies on the suppression of inflammation but also on minimising chronic morbidity (’damage’) is gaining ground. This involves surveillance for associated diseases such as malignancy, venous thromboembolic events and cardiovascular disease as well as minimising the risk of adverse drug effects such as renal toxicity.

• #49 Granulomatosis with Polyangiitis Rates & Outcomes – Page 3 of 3 – The Rheumatologist

  https://www.the-rheumatologist.org/article/granulomatosis-with-polyangiitis-rates-outcomes/3/

  Dr. White says epidemiological studies for rare diseases are key. Its important because with such rare diseases, it becomes difficult if not impossible to do randomized controlled trials prospectively, so you really need to try to look at the population as a whole. […] He says this study included only about 200 pediatric GPA cases, but that a researcher would perhaps want more than that for a randomized controlled trial. Epidemiology allows us to glean insights that would ordinarily not be obtainable.

• #50 Granulomatosis with Polyangiitis Rates & Outcomes – The Rheumatologist

  https://www.the-rheumatologist.org/article/granulomatosis-with-polyangiitis-rates-outcomes/

  A recent study in Arthritis Rheumatology highlights new information about the epidemiology and disease course of the vasculitic disease granulomatosis with polyangiitis (GPA, formerly known as Wegeners disease). GPA is a rare disease thats generally specific to the lungs, kidneys and the upper airways. The study provides key new data about the incidence and prevalence of the disease in the U.S. It also sheds light on disease course and outcomes. […] Information about GPA has been particularly limited for pediatric populations. Due to its relative rarity in children, much less has been known about the disease in this group, with studies limited to small cohorts. […] Overall, the researchers identified 5,566 cases of GPA. Of these, 3.8% (214) were pediatric cases. The incidence rate was 12.8 cases per million person-years in working-age adults (i.e., less than age 65), with a lower rate of 1.8 cases per million person-years for children.

• #51 Epidemiology and Outcomes of Granulomatosis with Polyangiitis (GPA) in Pediatric and Working-age Adults Populations in the United States: Analysis of a Large National Claims Database

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6258356/

  To our knowledge, our study represents the first large epidemiologic study of pediatric GPA, encompassing 214 pediatric GPA patients. We found an incidence rate of 1.8 per million person-years among children. […] The overall rate of ESRD appeared higher in pediatric patients compared to adults 65 years of age but this was not statistically significant. […] In summary, this large cohort of pediatric and working-age adult GPA patients provides novel information on incidence, prevalence, and outcomes of GPA in the United States.

• #52 Granulomatosis with Polyangiitis Rates & Outcomes – The Rheumatologist

  https://www.the-rheumatologist.org/article/granulomatosis-with-polyangiitis-rates-outcomes/

  The adult rate is comparable to that of a smaller report from the United Kingdom that reported a rate of 14.0 per million person-years. The pediatric rate was also in line with two other much smaller studies that found incidence rates of 2.8 and 0.9 cases per million person-years. […] The researchers also studied complication rates. Both children and working-age adults had high rates of hospitalizations and severe infections. We found that the most common complications were being hospitalized, having a severe infection and having hematologic abnormalities such as cytopenias, says Dr. White. We found that hospitalizations were a little more common in children. Of the severe infections, they were mostly of the respiratory tractpneumonias. The hematologic complications were more common in children than adults as well. In fact, children had two to three times higher rates of leukopenia, neutropenia and hypogammaglobulinemia. Pulmonary infections occurred at a rate of approximately 50 infections per 1,000 person-years in both groups.

• #53 Epidemiology and Outcomes of Granulomatosis with Polyangiitis (GPA) in Pediatric and Working-age Adults Populations in the United States: Analysis of a Large National Claims Database

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6258356/

  Given the paucity of epidemiology and outcome data of GPA in both adults and children, we performed a study using a large insurance claims dataset from the years 2006-2014 to identify and characterize both pediatric and adult GPA patients in the United States. […] The prevalence of GPA was 3.4 cases per million person-years in children and 30.5 cases per million person-years in adults less than 65 years of age. […] Our study demonstrated a slight female predominance in both pediatric and adult GPA cohorts, confirming the female predominance of pediatric GPA which has been observed in nearly all previous studies. […] Our study found an incidence rate of 12.8 per million person-years in the U.S. which is comparable with a prior smaller UK report which found an incidence rate of 14.0 per million person-years.

• #54 Granulomatosis with Polyangiitis Rates & Outcomes – The Rheumatologist

  https://www.the-rheumatologist.org/article/granulomatosis-with-polyangiitis-rates-outcomes/

  The adult rate is comparable to that of a smaller report from the United Kingdom that reported a rate of 14.0 per million person-years. The pediatric rate was also in line with two other much smaller studies that found incidence rates of 2.8 and 0.9 cases per million person-years. […] The researchers also studied complication rates. Both children and working-age adults had high rates of hospitalizations and severe infections. We found that the most common complications were being hospitalized, having a severe infection and having hematologic abnormalities such as cytopenias, says Dr. White. We found that hospitalizations were a little more common in children. Of the severe infections, they were mostly of the respiratory tractpneumonias. The hematologic complications were more common in children than adults as well. In fact, children had two to three times higher rates of leukopenia, neutropenia and hypogammaglobulinemia. Pulmonary infections occurred at a rate of approximately 50 infections per 1,000 person-years in both groups.

• #55 Epidemiology and Outcomes of Granulomatosis with Polyangiitis (GPA) in Pediatric and Working-age Adults Populations in the United States: Analysis of a Large National Claims Database

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6258356/

  To our knowledge, our study represents the first large epidemiologic study of pediatric GPA, encompassing 214 pediatric GPA patients. We found an incidence rate of 1.8 per million person-years among children. […] The overall rate of ESRD appeared higher in pediatric patients compared to adults 65 years of age but this was not statistically significant. […] In summary, this large cohort of pediatric and working-age adult GPA patients provides novel information on incidence, prevalence, and outcomes of GPA in the United States.

• #56 Granulomatosis With Polyangiitis | Applied Radiology

  https://www.appliedradiology.com/articles/granulomatosis-with-polyangiitis

  Granulomatosis with polyangiitis (GPA) is a rare multisystem, autoimmune disease of unknown etiology characterized by granulomatous, necrotizing lesions affecting small- to medium-sized blood vessels. It most commonly affects middle-aged women. […] GPA is more commonly diagnosed in adults than in children; prevalence in the pediatric population ranges from 0.45-6.39 cases/million/year depending on the referenced study. […] Panupattanapong, et al, reported the largest cohort of patients with GPA to date (n=5,562). In their study, 3.8% of all patients had pediatric-onset disease. […] There are no specific guidelines for the management of GPA in children. Thus, treatment is extrapolated from adult management and expert consensus. […] Granulomatosis with polyangiitis, while more commonly identified in adults, should be suspected in children presenting with hemoptysis, hematuria, and chest imaging showing cavitary pulmonary nodules, ground-glass opacity, and consolidation.

• #57 The eye is a common site of granulomatosis with polyangiitis. A collaborative study | BMC Ophthalmology | Full Text

  https://bmcophthalmol.biomedcentral.com/articles/10.1186/s12886-022-02743-x

  Ocular manifestations of granulomatosis with polyangiitis (GPA) have been reported in a limited number of studies and with largely variable frequency. Here we report on the clinical, diagnostic, and therapeutic features of a cohort of 63 GPA patients, with particular regard to 22 of them with ophthalmic involvement (35%). […] While there are substantial differences in the incidence rate of GPA in Europe and Asia and as a function of latitude, the overall incidence rate of the disease is 0.4-1.9 cases/million person-years, with a prevalence of 2.3-146.0 cases/million persons. Epidemiological studies, however, have documented a progressive increase in GPA, reflecting a wider awareness of the disease and the increased availability of reliable imaging techniques. In a population-based cohort study carried out in Denmark, the median annual incidence rate was 20.5/million persons, with an increase in the point prevalence from 64/million in 1995 to 277/million in 2015.

• #58 Epidemiology and Treatment Outcome of ANCA-associated Vasculitis in South Korea: A Nationwide, Population-based Cohort Study – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/epidemiology-and-treatment-outcome-of-anca-associated-vasculitis-in-south-korea-a-nationwide-population-based-cohort-study/

  Results: A total of 546 and 795 patients with GPA and MPA, respectively, were identified. The incidence rates of both diseases increased with age, and the peak incidence rate was observed in patients aged 70 years. GPA and MPA showed different trends in incidence rates over time. In 2010, the annual incidence rates (per million) of GPA and MPA were 1.03 and 0.83, respectively. The incidence of MPA has continuously increased over time and increased to 3.26 in 2018. In contrast, the annual incidence of GPA did not significantly change during the observation period and was 1.33 in 2018. […] During the observation period, 90 (28.3%) and 187 (38.6%) patients in the GPA and MPA groups, respectively, died. Although all-cause mortality rates in the GPA and MPA group were significantly higher compared to that in each comparator group (adjusted HR 5.15 [3.21 to 8.38] and 8.62 [6.52 to 11.4], respectively), it was even higher in patients with MPA (adjusted HR 1.69 [1.31 to 2.18]). Patients in the GPA and MPA groups also showed a significantly higher risk of renal replacement therapy and ICU admission than those in the comparator population. In both groups, mortality and morbidity outcomes occurred mainly within the first year from the diagnosis of underlying vasculitis. […] Conclusion: In South Korea, the incidence of MPA has increased over time. Although both GPA and MPA had high rates of mortality and morbidity, MPA has a poorer prognosis than GPA.

• #59 Granulomatosis with polyangiitis in Northeastern Brazil: study of 25 cases and review of the literature | Advances in Rheumatology | Full Text

  https://advancesinrheumatology.biomedcentral.com/articles/10.1186/s42358-018-0010-3

  Little has been published about the epidemiology of Granulomatosis with polyangiitis (GPA) in South America, especially in the intertropical zone, and no epidemiological data from Brazil are available. […] The purpose of the present study was to draw a clinical and demographic profile of GPA patients living in Northeastern Brazil based on laboratory, histological and imaging findings, and evaluate the frequency of organic involvement in relation to data from the international literature. […] The 2016 prevalence of GPA in the metropolitan region of Fortaleza was estimated at 5.0/1,000,000 pop. […] The observed differences in the frequency of certain types of clinical manifestations (e.g., ENT and pulmonary) between Northeastern Brazil and other regions in the northern and southern hemispheres may be explained by the influence of environmental and latitudinal factors on clinical phenotype.

• #60 Granulomatosis with polyangiitis in Northeastern Brazil: study of 25 cases and review of the literature | Advances in Rheumatology | Full Text

  https://advancesinrheumatology.biomedcentral.com/articles/10.1186/s42358-018-0010-3

  In this study from Northeastern Brazil, most patients were female, had the generalized form of the disease, presented nephritic syndrome during the evolution of the disease and were diagnosed late. Overall, the frequency of clinical manifestations was comparable to that reported in the international literature, with some differences in relation to the northern hemisphere (pulmonary and renal involvement) and the southern hemisphere (ENT involvement), suggesting the existence of a Northeast Brazilian clinical and epidemiological profile and adding to our knowledge of this rare condition.

• #61 Too early diagnosis of granulomatosis with polyangiitis (GPA) in the first month of initial presentation

  https://www.probiologists.com/article/too-early-diagnosis-of-granulomatosis-with-polyangiitis-gpa-in-the-first-month-of-initial-presentation

  Granulomatosis with Polyangiitis (GPA) is a systemic small sized vascular disease involving vasculitis, granuloma, and necrosis that most often targets the Ear, Nose and Throat (ENT) and/or Lungs and/or Kidneys, and it is classically associated with Anti-Neutrophil Cytoplasmic Antibody (ANCA) positivity. […] The prevalence of GPA in the world varies from 2 in 100,000 to 5 in 100,000, which according to the author of this article, the prevalence of 3 in 100,000, and annual incidence of 1 in 100,000 are the best options. […] Currently, the interval between the onset of GPA and its definitive diagnosis is between 2 and 20 months. […] Well-known Criteria have been presented for Classifying/Diagnosing Wegener’s Granulomatosis or GPA that are: „The 1990 American College of Rheumatology (ACR) Classification Criteria for Wegener’s Granulomatosis”, „The 2007 European Medicine Agency Algorithm (EMA) Diagnostic Criteria for Systemic GPA”, and „The ACR/European League Against Rheumatism (EULAR) Provisional 2017 Classification Criteria for GPA”.

• #62 Too early diagnosis of granulomatosis with polyangiitis (GPA) in the first month of initial presentation

  https://www.probiologists.com/article/too-early-diagnosis-of-granulomatosis-with-polyangiitis-gpa-in-the-first-month-of-initial-presentation

  The Iran Criteria for Early Diagnosis of GPA suggests evaluation of three organs and two laboratory findings (ANCA and biopsy), after ruling out other prominent diagnoses by history and physical examination. […] The 2017 ACR/EMA Revised Criteria for Too Early Diagnosis of GPA was named. […] It is claimed that they are able to diagnose GPA in the first a few months and the first month of the disease, respectively. […] By application of The Iran Criteria for Early Diagnosis of GPA only about 50% of our cases with GPA became systemic, due to the earlier diagnosis and management of GPA. […] You have to know that with a little change within Iran Criteria a new Criteria is created by the author of this letter called the 2017 ACR/EMA Revised Criteria for Too Early Diagnosis of GPA by which the diagnosis of GPA can be established earlier within the first month of initial presentation of the disease.

• #63 Inpatient Epidemiology of Granulomatosis with Polyangiitis in the United States – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/inpatient-epidemiology-of-granulomatosis-with-polyangiitis-in-the-united-states/

  The inpatient prevalence of GPA was higher than what would be expected from the overall incidence. Hospitalizations of these patients were associated with high morbidity and mortality. The mean total hospital costs, charges, and LOS for patients admitted with GPA were higher than patients without GPA.

• #64 Inpatient Epidemiology of Granulomatosis with Polyangiitis in the United States – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/inpatient-epidemiology-of-granulomatosis-with-polyangiitis-in-the-united-states/

  Inpatient Epidemiology of Granulomatosis with Polyangiitis in the United States […] Granulomatosis with polyangiitis (GPA, formerly known and Wegeners granulomatosis) is a major subtype of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis characterized by granulomatous inflammation of small- and medium- size vessels. Data on inpatient epidemiology, resource utilization, and healthcare expenditures of GPA are not well described. The aim of this study was to explore those characteristics using a large national inpatient database. […] A total of 124,682 admissions of patients with a diagnosis of GPA occurred in the study period. The mean age was 60.2 years and 52.3% were female. For the primary outcome, the inpatient prevalence of GPA was 32.6 cases per 100,000 discharges.

• #65 Inpatient Epidemiology of Granulomatosis with Polyangiitis in the United States – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/inpatient-epidemiology-of-granulomatosis-with-polyangiitis-in-the-united-states/

  The inpatient prevalence of GPA was higher than what would be expected from the overall incidence. Hospitalizations of these patients were associated with high morbidity and mortality. The mean total hospital costs, charges, and LOS for patients admitted with GPA were higher than patients without GPA.

• #66 Inpatient Epidemiology of Granulomatosis with Polyangiitis in the United States – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/inpatient-epidemiology-of-granulomatosis-with-polyangiitis-in-the-united-states/

  The inpatient prevalence of GPA was higher than what would be expected from the overall incidence. Hospitalizations of these patients were associated with high morbidity and mortality. The mean total hospital costs, charges, and LOS for patients admitted with GPA were higher than patients without GPA.

• #67 Granulomatosis with Polyangiitis Rates & Outcomes – Page 3 of 3 – The Rheumatologist

  https://www.the-rheumatologist.org/article/granulomatosis-with-polyangiitis-rates-outcomes/3/

  Dr. White says epidemiological studies for rare diseases are key. Its important because with such rare diseases, it becomes difficult if not impossible to do randomized controlled trials prospectively, so you really need to try to look at the population as a whole. […] He says this study included only about 200 pediatric GPA cases, but that a researcher would perhaps want more than that for a randomized controlled trial. Epidemiology allows us to glean insights that would ordinarily not be obtainable.

• #68 Granulomatosis With Polyangiitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557827/

  Epidemiology: Among the 3 ANCA-associated vasculitides, GPA is the most common. The annual worldwide incidence of GPA is estimated to be 10 to 20 cases per million based on the geographical location. A higher incidence is noted in the colder regions. The prevalence of GPA in European and American populations is about 120 to 140 per million. A national study in the Netherlands on ANCA-associated vasculitis found that 167 patients (73%) were diagnosed with GPA, 54 (24%) with MPA, and 9 (4%) with EGPA. This distribution is similar to other European registries. […] GPA is more commonly reported in Whites, although it can be observed in all racial and ethnic groups. The onset of GPA occurs between 45 and 60 years, but a small proportion (3%-7%) affects children and adolescents. Children younger than 18 have a female-to-male predominance of about 2:1, while in adults, the ratio is 1:1.

• #69 Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis): Practice Essentials, Background, Etiology

  https://emedicine.medscape.com/article/332622-overview

  GPA is a rare disease with an as yet undetermined incidence. The prevalence of GPA in the United States is estimated to be 3 cases per 100,000 people. […] The incidence and prevalence of GPA in the United Kingdom is estimated at 10.2 cases and 250 cases per million population, respectively. […] GPA is more common in individuals of northern European descent (approximately 90%); it occurs less commonly in blacks. […] In European populations, GPA is slightly more common in men, with a male-to-female ratio of 1.5:1. […] The onset of GPA may occur at any age, although patients typically present at age 35-55 years. GPA is rare in childhood.

• #70 Epidemiology and Outcomes of Granulomatosis with Polyangiitis (GPA) in Pediatric and Working-age Adults Populations in the United States: Analysis of a Large National Claims Database

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6258356/

  Given the paucity of epidemiology and outcome data of GPA in both adults and children, we performed a study using a large insurance claims dataset from the years 2006-2014 to identify and characterize both pediatric and adult GPA patients in the United States. […] The prevalence of GPA was 3.4 cases per million person-years in children and 30.5 cases per million person-years in adults less than 65 years of age. […] Our study demonstrated a slight female predominance in both pediatric and adult GPA cohorts, confirming the female predominance of pediatric GPA which has been observed in nearly all previous studies. […] Our study found an incidence rate of 12.8 per million person-years in the U.S. which is comparable with a prior smaller UK report which found an incidence rate of 14.0 per million person-years.

• #71 Granulomatosis with Polyangiitis (GPA) – Musculoskeletal and Connective Tissue Disorders – Merck Manual Professional Edition

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

  Prognosis depends on the severity and extent of disease and at least as much on how rapidly treatment occurs. Use of immunosuppressants for severe disease has dramatically improved prognosis. With treatment, complete remission is possible for approximately 80% of patients, but approximately half of them eventually relapse.

• #72 Score to assess the probability of relapse in granulomatosis with polyangiitis and microscopic polyangiitis | RMD Open

  https://rmdopen.bmj.com/content/9/1/e002953

  Granulomatosis with polyangiitis and microscopic polyangiitis are two antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) in which relapse is common. There is a lack of criteria to predict the risk of relapse in AAV. […] We identified three factors (PR3-ANCA, age 75 years and estimated glomerular filtration rate 30mL/min/1.73m) associated with a higher risk of relapse of AAV. These factors were combined to form a score, ranging from 0 to 3 points (1 point for each factor) that predicts the risk of relapse in AAV. […] This score could, therefore, help clinicians tailor the duration of maintenance therapy in AAV, provided it is evaluated and validated in future prospective trials. […] The objective of this study was to identify baseline clinical and biological patient characteristics associated with the probability of relapse during follow-up in order to develop a relapse prediction score. This score could help clinicians determine whether patients in remission after induction therapy should receive extended maintenance treatment.

• #73 Score to assess the probability of relapse in granulomatosis with polyangiitis and microscopic polyangiitis | RMD Open

  https://rmdopen.bmj.com/content/9/1/e002953

  A higher risk of relapse was independently associated with PR3-ANCA (HR=1.81 (95% CI 1.28 to 2.57); p0.001), age 75 years (HR=1.89 (95% CI 1.15 to 3.13); p=0.012) and eGFR (CKD-EPI) 30mL/min/1.73m (HR=1.67 (95% CI 1.18 to 2.33); p=0.004) at AAV diagnosis. […] This study provides a simple score, entitled FRS, which can be calculated at the time of diagnosis of GPA or MPA to predict the risk of relapse during follow-up and which may ultimately allow clinicians to tailor the duration of maintenance therapy for these vasculitides. […] The FRS is not applicable to EGPA and PAN patients, who were not included in this study. In PAN, the risk of relapse is lower. […] Our study has many strengths: the reliability of the data (data from prospective therapeutic trials), the use of a Fine-Gray model to distinguish between two competitive risks (death and relapse), the analysis stratified on the induction and maintenance treatment (which influences the progression of the disease), and its validation in an independent cohort, which allowed us to show that the FRS reliably predicts the risk of relapse and that its level of performance increases with time.

• #74 Too early diagnosis of granulomatosis with polyangiitis (GPA) in the first month of initial presentation

  https://www.probiologists.com/article/too-early-diagnosis-of-granulomatosis-with-polyangiitis-gpa-in-the-first-month-of-initial-presentation

  The Iran Criteria for Early Diagnosis of GPA suggests evaluation of three organs and two laboratory findings (ANCA and biopsy), after ruling out other prominent diagnoses by history and physical examination. […] The 2017 ACR/EMA Revised Criteria for Too Early Diagnosis of GPA was named. […] It is claimed that they are able to diagnose GPA in the first a few months and the first month of the disease, respectively. […] By application of The Iran Criteria for Early Diagnosis of GPA only about 50% of our cases with GPA became systemic, due to the earlier diagnosis and management of GPA. […] You have to know that with a little change within Iran Criteria a new Criteria is created by the author of this letter called the 2017 ACR/EMA Revised Criteria for Too Early Diagnosis of GPA by which the diagnosis of GPA can be established earlier within the first month of initial presentation of the disease.

• #75 Score to assess the probability of relapse in granulomatosis with polyangiitis and microscopic polyangiitis | RMD Open

  https://rmdopen.bmj.com/content/9/1/e002953

  Granulomatosis with polyangiitis and microscopic polyangiitis are two antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) in which relapse is common. There is a lack of criteria to predict the risk of relapse in AAV. […] We identified three factors (PR3-ANCA, age 75 years and estimated glomerular filtration rate 30mL/min/1.73m) associated with a higher risk of relapse of AAV. These factors were combined to form a score, ranging from 0 to 3 points (1 point for each factor) that predicts the risk of relapse in AAV. […] This score could, therefore, help clinicians tailor the duration of maintenance therapy in AAV, provided it is evaluated and validated in future prospective trials. […] The objective of this study was to identify baseline clinical and biological patient characteristics associated with the probability of relapse during follow-up in order to develop a relapse prediction score. This score could help clinicians determine whether patients in remission after induction therapy should receive extended maintenance treatment.

• #76 Score to assess the probability of relapse in granulomatosis with polyangiitis and microscopic polyangiitis | RMD Open

  https://rmdopen.bmj.com/content/9/1/e002953

  A higher risk of relapse was independently associated with PR3-ANCA (HR=1.81 (95% CI 1.28 to 2.57); p0.001), age 75 years (HR=1.89 (95% CI 1.15 to 3.13); p=0.012) and eGFR (CKD-EPI) 30mL/min/1.73m (HR=1.67 (95% CI 1.18 to 2.33); p=0.004) at AAV diagnosis. […] This study provides a simple score, entitled FRS, which can be calculated at the time of diagnosis of GPA or MPA to predict the risk of relapse during follow-up and which may ultimately allow clinicians to tailor the duration of maintenance therapy for these vasculitides. […] The FRS is not applicable to EGPA and PAN patients, who were not included in this study. In PAN, the risk of relapse is lower. […] Our study has many strengths: the reliability of the data (data from prospective therapeutic trials), the use of a Fine-Gray model to distinguish between two competitive risks (death and relapse), the analysis stratified on the induction and maintenance treatment (which influences the progression of the disease), and its validation in an independent cohort, which allowed us to show that the FRS reliably predicts the risk of relapse and that its level of performance increases with time.

• #77 Too early diagnosis of granulomatosis with polyangiitis (GPA) in the first month of initial presentation

  https://www.probiologists.com/article/too-early-diagnosis-of-granulomatosis-with-polyangiitis-gpa-in-the-first-month-of-initial-presentation

  The Iran Criteria for Early Diagnosis of GPA suggests evaluation of three organs and two laboratory findings (ANCA and biopsy), after ruling out other prominent diagnoses by history and physical examination. […] The 2017 ACR/EMA Revised Criteria for Too Early Diagnosis of GPA was named. […] It is claimed that they are able to diagnose GPA in the first a few months and the first month of the disease, respectively. […] By application of The Iran Criteria for Early Diagnosis of GPA only about 50% of our cases with GPA became systemic, due to the earlier diagnosis and management of GPA. […] You have to know that with a little change within Iran Criteria a new Criteria is created by the author of this letter called the 2017 ACR/EMA Revised Criteria for Too Early Diagnosis of GPA by which the diagnosis of GPA can be established earlier within the first month of initial presentation of the disease.

• #78 Score to assess the probability of relapse in granulomatosis with polyangiitis and microscopic polyangiitis | RMD Open

  https://rmdopen.bmj.com/content/9/1/e002953

  Granulomatosis with polyangiitis and microscopic polyangiitis are two antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) in which relapse is common. There is a lack of criteria to predict the risk of relapse in AAV. […] We identified three factors (PR3-ANCA, age 75 years and estimated glomerular filtration rate 30mL/min/1.73m) associated with a higher risk of relapse of AAV. These factors were combined to form a score, ranging from 0 to 3 points (1 point for each factor) that predicts the risk of relapse in AAV. […] This score could, therefore, help clinicians tailor the duration of maintenance therapy in AAV, provided it is evaluated and validated in future prospective trials. […] The objective of this study was to identify baseline clinical and biological patient characteristics associated with the probability of relapse during follow-up in order to develop a relapse prediction score. This score could help clinicians determine whether patients in remission after induction therapy should receive extended maintenance treatment.

• #79 Score to assess the probability of relapse in granulomatosis with polyangiitis and microscopic polyangiitis | RMD Open

  https://rmdopen.bmj.com/content/9/1/e002953

  A higher risk of relapse was independently associated with PR3-ANCA (HR=1.81 (95% CI 1.28 to 2.57); p0.001), age 75 years (HR=1.89 (95% CI 1.15 to 3.13); p=0.012) and eGFR (CKD-EPI) 30mL/min/1.73m (HR=1.67 (95% CI 1.18 to 2.33); p=0.004) at AAV diagnosis. […] This study provides a simple score, entitled FRS, which can be calculated at the time of diagnosis of GPA or MPA to predict the risk of relapse during follow-up and which may ultimately allow clinicians to tailor the duration of maintenance therapy for these vasculitides. […] The FRS is not applicable to EGPA and PAN patients, who were not included in this study. In PAN, the risk of relapse is lower. […] Our study has many strengths: the reliability of the data (data from prospective therapeutic trials), the use of a Fine-Gray model to distinguish between two competitive risks (death and relapse), the analysis stratified on the induction and maintenance treatment (which influences the progression of the disease), and its validation in an independent cohort, which allowed us to show that the FRS reliably predicts the risk of relapse and that its level of performance increases with time.

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