Materiały źródłowe

• #1 Global epidemiology of vasculitis | Nature Reviews Rheumatology

  https://www.nature.com/articles/s41584-021-00718-8

  The many forms of vasculitis are characterized by inflammation of blood vessels, leading to potentially long-term sequelae including vision loss, aneurysm formation and kidney failure. Accurate estimation of the incidence and prevalence has been hampered by the absence of reliable diagnostic criteria and the rarity of these conditions; however, much progress has been made over the past two decades, although data are still lacking from many parts of the world including the Indian subcontinent, China, Africa and South America. […] Although much less common than giant cell arteritis, the different forms of antineutrophil cytoplasmic antibody-associated vasculitis are being increasingly recognized in most populations and occur more frequently with increasing age. […] Much work needs to be done to better understand the influence of ethnicity, geographical location, environment and social factors on the development of vasculitis.

• #2 Global epidemiology of vasculitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8633913/

  The many forms of vasculitis are characterized by inflammation of blood vessels, leading to potentially long-term sequelae including vision loss, aneurysm formation and kidney failure. Accurate estimation of the incidence and prevalence has been hampered by the absence of reliable diagnostic criteria and the rarity of these conditions; however, much progress has been made over the past two decades, although data are still lacking from many parts of the world including the Indian subcontinent, China, Africa and South America. […] Although much less common than giant cell arteritis, the different forms of antineutrophil cytoplasmic antibody-associated vasculitis are being increasingly recognized in most populations and occur more frequently with increasing age. […] Reliable classification criteria are an absolute requirement for conducting accurate epidemiology, yet there has been no universal system of classification for vasculitis.

• #3 Global epidemiology of vasculitis.

  https://www.repository.cam.ac.uk/items/43d088bb-2c82-4f74-8756-11cb49331a23

  The many forms of vasculitis are characterized by inflammation of blood vessels, leading to potentially long-term sequelae including vision loss, aneurysm formation and kidney failure. Accurate estimation of the incidence and prevalence has been hampered by the absence of reliable diagnostic criteria and the rarity of these conditions; however, much progress has been made over the past two decades, although data are still lacking from many parts of the world including the Indian subcontinent, China, Africa and South America. […] Much work needs to be done to better understand the influence of ethnicity, geographical location, environment and social factors on the development of vasculitis.

• #4 Vasculitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK545186/

  The overall incidence per annum of primary systemic vasculitis is approximately 20 to 40/million, according to studies in Europe and the USA. […] GCA is the most common among the primary vasculitides, with an annual incidence (in the population of individuals at least 50 years old) of around 240 per million. […] Granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and Churg-Strauss syndrome have been reported at an incidence anywhere from 1 to 10 per million.

• #5 Vasculitis Diagnostics

  https://www.diagnostic.grifols.com/en/-/learning/vasculitis-diagnostics

  Overall, the incidence (number of new cases per year) of vasculitis has been reported as approximately 2054 cases per 1 million population, but this varies widely. […] A recent UK study reported the incidence of temporal arteritis as 130300 per million in individuals aged over 50 years. […] The incidence of Kawasaki disease was reported as 5584 per million in children aged under 5 years. […] The overall annual incidence of AAV has been reported with wide-ranging values of approximately 1033 per million. […] Considerable geographic variation exists in the occurrence of AAV. […] Nonetheless, in Asia, the most common AAV appears to be MPA that is positive for myeloperoxidase enzyme (MPO). […] In Northern Europe and the USA, the most common AAV appears to be GPA that is positive for leukocyte proteinase 3 (PR3).

• #6

  https://step1.medbullets.com/evidence/10693883

  Epidemiology of systemic vasculitis: a ten-year study in the United Kingdom. […] To describe the epidemiology of the primary systemic vasculitides (PSV; Wegener’s granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, polyarteritis nodosa) in a well-defined population over a 10-year period. […] Eighty-two NHA residents fulfilled the inclusion criteria. There were 47 men and 35 women, with a mean age of 62.9 years (median 65.0 years). The overall annual incidence of PSV among NHA residents was 19.8/million (95% confidence interval [95% CI] 15.8-24.6). The point prevalence on December 31, 1997 was 144.5/million (95% CI 110.4-185.3). PSV was more common in males (23.5/million; 95% CI 17.3-31.3) than females (16.4/million; 95% CI 11.4-22.8). The age- and sex-specific incidence showed a clear increase with age, with an overall peak in the 65-74 year age group (60.1/million). […] In our study population, the annual incidence of PSV is slowly increasing with time and the incidence is greatest in the elderly.

• #7 Global epidemiology of vasculitis | Nature Reviews Rheumatology

  https://www.nature.com/articles/s41584-021-00718-8

  Vasculitis is a group of generally uncommon diseases of generally unknown aetiology. […] Vasculitis mainly occurs at the ends of the age spectrum, in young children and older adults. […] The three types of antineutrophil cytoplasmic antibody-associated vasculitis are rare; of the three types, granulomatosis with polyangiitis is the most prevalent. […] Behet syndrome has the highest prevalence along the ancient silk road, which stretches from the Mediterranean through the Middle East to East Asia.

• #8 Global epidemiology of vasculitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8633913/

  Two main types of study have been used to assess the incidence and prevalence of vasculitis: cohort studies and registry studies. […] Reliable data are therefore lacking from many regions, particularly Africa, the Indian subcontinent, China and Latin America. Importantly, this lack of data does not mean that the conditions do not occur in these locations, just that the tools for measuring disease occurrence and burden are not available. […] In conclusion, knowledge of the epidemiology of vasculitis has increased over the past two decades, and it has become apparent that vasculitis occurs most commonly at the ends of the age spectrum.

• #9

  https://journals.lww.com/co-rheumatology/fulltext/2024/01000/epidemiology_of_systemic_vasculitis.6.aspx

  Epidemiology of vasculitides exhibit geographic variation and data from some parts of the world are still scarce. […] Increased recognition of these rare diseases and improvement in diagnosis and patient care may lead to changes in their epidemiology. […] New data from countries where information on the epidemiology of giant cell arteritis, Takayasu arteritis and Behet syndrome were limited have revealed that these conditions are not as rare as previously believed. […] The use of different classification criteria hampers the comparison of true incidence and prevalence rates in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis and its subtypes between geographies and over time. […] Recent studies have highlighted the epidemiology of vasculitides in different parts of the world and changing trends. Standardization of study design and disease definitions is needed to improve the reliability and comparability of the results.

• #10 Epidemiology of systemic vasculitis: Ingenta Connect

  https://www.ingentaconnect.com/content/wk/bor/2024/00000036/00000001/art00006

  Epidemiology of vasculitides exhibit geographic variation and data from some parts of the world are still scarce. […] New data from countries where information on the epidemiology of giant cell arteritis, Takayasu arteritis and Behet syndrome were limited have revealed that these conditions are not as rare as previously believed. […] The use of different classification criteria hampers the comparison of true incidence and prevalence rates in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis and its subtypes between geographies and over time. […] Recent studies have highlighted the epidemiology of vasculitides in different parts of the world and changing trends. Standardization of study design and disease definitions is needed to improve the reliability and comparability of the results.

• #11

  https://www.ntnu.no/ojs/index.php/norepid/article/view/62

  Nonetheless there are interesting differences in the prevalence of specific vasculitis between different geographical areas, as well as for sub specificities of ANCA. […] There seems to be a South-North gradient for WG and PR3-ANCA with high figures reported from northern Europe and southern New Zealand. […] MPA which is predominantly MPO-ANCA associated are more frequent in the Mediterranean countries and also has an increasing gradient towards east-Asia, as almost all AAV in China and Japan are diagnosed as MPA, predominantly MPO-ANCA positive. […] There are also some ethnic and gender differences. WG is most prevalent among Caucasians in the USA and in people with European ancestors in Paris and in New Zealand, less frequent in Africans and Asians. […] Several studies have shown that the highest incidence of WG is in males 60-70 years old. Females are younger at onset, and in children WG is most frequent in girls. […] With better treatment (cyclophosphamide and corticosteroids), the survival of AAV has increased considerably. However disease control is not optimal, as most of the vasculitis present a remitting-relapsing course and organ damage is considerable.

• #12 Vasculitis | Diagnosis & Disease Info – Rheumatology Advisor

  https://www.rheumatologyadvisor.com/ddi/vasculitis/

  The annual incidence of Kawasaki disease in the United States is estimated to be 20 per 100,000 children under 5 years of age and more likely to be seen in boys than girls. Children of Asian or Pacific Islander descent have a higher incidence of the disease. […] The incidence of AAV is approximately 33.0 per million individuals in the United States. The incidence has increased in the last 40 years, likely due to improved and widespread testing. Individuals of Japanese, Chinese, and Southern European descent have an increased risk of being diagnosed with MPA compared with individuals of Northern European descent. […] The annual incidence of Behet syndrome ranges from 0.05 to 3.9 per 100,000 individuals depending on the country. The highest prevalence was observed along the ancient Silk Road, ranging from 10.0 per 100,000 individuals in China to 421 per 100,000 individuals in Turkey. In the United States, the prevalence of Behet syndrome is between 0.33 and 10.6 per 100,000 individuals.

• #13 Epidemiology and management of interstitial lung disease in ANCA-associated vasculitis

  https://www.clinexprheumatol.org/abstract.asp?a=15448

  Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a group of systemic vasculitides that predominantly affect small vessels, including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). […] Prevalence of ILD is higher in MPA than in GPA; in fact, ILD has been reported in up to 45% of MPA patients and in 23% of GPA. […] High resolution computed tomography (HRCT) frequently detects interstitial lung abnormalities in AAV, up to 66% of patients with MPA, even if with an unclear clinical relevance, specifically in asymptomatic patients. […] ILD significantly affects quality of life and survival, with mortality increased 2 to 4 times, particularly higher in MPA patients with pulmonary fibrosis. […] Currently, immunosuppressive therapy is considered also as a possible treatment of ILD.

• #14 ANCA Associated Vasculitis Industry & Epidemiology Analysis

  https://www.globenewswire.com/news-release/2023/07/21/2708731/0/en/ANCA-Associated-Vasculitis-Industry-Epidemiology-Analysis-and-Forecast-2032-Includes-Insights-Into-Drugs-and-Key-Players.html

  The report delivers an in-depth understanding of the ANCA Associated Vasculitis, historical and forecasted epidemiology as well as the ANCA Associated Vasculitis market trends in the United States, EU4 (Germany, France, Italy, and Spain), and the United Kingdom, Japan. […] The total diagnosed prevalent cases of ANCA-associated vasculitis in the 7MM comprised approximately 207,900 cases in 2022 and are projected to increase during the forecasted period. […] The total diagnosed prevalent cases of ANCA-associated vasculitis in the United States were around 70,700 cases in 2022. […] In the 7MM, the United States, EU4, the UK, and Japan accounted for around 34%, 42%, and 24% of the total diagnosed prevalent population share in 2022. […] According to the estimates, Japan had around 23,000, 17,700, and 9,420 diagnosed prevalent cases of MPA, GPA, and EGPA, respectively, in 2022. These cases are projected to increase during the forecasted period.

• #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/

  Epidemiology and Treatment Outcome of ANCA-associated Vasculitis in South Korea: A Nationwide, Population-based Cohort Study […] In this study, we investigated the epidemiological features of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) in South Korea. […] 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. […] 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 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 RINVOQ® (upadacitinib) Receives U.S. FDA Approval for Giant Cell Arteritis (GCA) – Apr 29, 2025

  https://news.abbvie.com/2025-04-29-RINVOQ-R-upadacitinib-Receives-U-S-FDA-Approval-for-Giant-Cell-Arteritis-GCA

  GCA is the most common vasculitis affecting adults in Western countries. […] Giant cell arteritis (GCA), also known as temporal arteritis, is an autoimmune disease of medium and large arteries, characterized by granulomatous inflammation of the three-layered vessel wall, which affects temporal and other cranial arteries as well as the aorta and other large arteries. GCA can cause headache, jaw pain, and changes in or loss of vision, including sudden and permanent loss of vision. It is the most common vasculitis affecting adults in Western countries.

• #17 Vasculitis | Diagnosis & Disease Info – Rheumatology Advisor

  https://www.rheumatologyadvisor.com/ddi/vasculitis/

  Vasculitis as a whole affects approximately 38 to 40 people per million and is generally more common in adults than in children. Accurate estimation of incidence and prevalence is hindered by the rarity of certain forms of vasculitis. […] The highest incidence of GCA is seen in Scandinavian (21.6 per 100,000 individuals) and Northern American (10.89 per 100,000 individuals) populations. In North America, data from Olmsted County, Minnesota, suggest that the prevalence of GCA is 204 per 100,000 individuals. The prevalence of Takayasu arteritis is approximately 8.4 per million individuals in the United States. […] Hepatitis B is a potential trigger for PAN and this form of vasculitis may occur in association with familial Mediterranean fever. In European countries, the annual incidence rate is between 0.9 and 8.0 per million individuals and prevalence rate is approximately 31 per million individuals.

• #18 Vasculitis | Diagnosis & Disease Info – Rheumatology Advisor

  https://www.rheumatologyadvisor.com/ddi/vasculitis/

  The exact cause of vasculitis remains unknown. Various risk factors have been linked with an increase in incidence and prevalence of the disease. […] These include age, geography, gender, and ethnicity along with genetic and environmental factors. Hepatitis B and C infection can trigger immune system disorders such as rheumatoid arthritis, lupus, or scleroderma and have also been implicated in disease risk. […] GCA is the most common systemic vasculitis in patients older than 50 years of age while Kawasaki disease is more prevalent in children younger than 5 years of age. Human leukocyte antigen (HLA)-DRB1*04 is associated with GCA, HLA-B51/55 is associated with Behet disease, and Kawasaki disease has numerous genetic associations. Behet disease shows increased severity among male patients, whereas Takayasu arteritis has a female preponderance of 9 to 1.

• #19 Incidence of vasculitis in children in the Czech Republic: 2-year prospective epidemiology survey. | The Journal of Rheumatology

  https://www.jrheum.org/content/31/11/2295

  OBJECTIVE: To determine the incidence and presenting features of primary and secondary vasculitides in children across the Czech Republic. […] We identified 452 new cases of vasculitis and connective tissue disease. The estimated annual incidence of Henoch-Schonlein purpura (HSP) was 10.2/100,000 children, with a mean age at onset of 7 years. […] The estimated annual incidence of Kawasaki disease (KD) was 1.6/100,000 children under 5 years. […] Secondary vasculitides of connective tissue diseases had an estimated annual incidence of 0.22/100,000 for systemic lupus erythematosus and 0.19/100,000 for dermatomyositis. […] We determined the incidence of different childhood vasculitides within a hospital based population throughout the Czech Republic. HSP was the most common, with a relatively high proportion of the patients treated with a short course of corticosteroids. A lower incidence than expected of KD raised the suspicion that some cases were not identified. Other childhood vasculitides were rare.

• #20 Henoch-Schönlein Purpura (IgA Vasculitis): Rapid Evidence Review | AAFP

  https://www.aafp.org/pubs/afp/issues/2020/0815/p229.html

  IgA vasculitis occurs in 3.0 to 26.7 out of 100,000 children and 0.8 to 1.8 out of 100,000 adults each year.1 […] The mean age of onset in children is six years; onset ranged from 32 to 50 years of age in several adult case series.14 More than 90% of cases occur in children younger than 10 years.3 […] There is a slight disease predominance in males.1,2 […] In children, onset is more common during the fall and winter, but no seasonal pattern has been consistently shown in adults.1,5 […] IgA vasculitis is milder in children younger than two years, but more severe in adults, with worse outcomes.3,4,6

• #21 Small-Vessel Vasculitis (Leukocytoclastic Vasculitis): Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/333891-overview

  SVV with skin predominance (LCV) is reported to occur in 10-30 persons per million annually. Many of the estimates come from Spanish cohorts. […] Cutaneous LCV has been reported more frequently in males.

• #22 Hypersensitivity Vasculitis: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1083719-overview

  The incidence of hypersensitivity vasculitis is unknown, but the condition is presumed to be relatively rare. A 2014 population-based study in Minnesota found the incidence of cutaneous leukocytoclastic vasculitis in adults (including IgA vasculitis as well as other types of small-vessel vasculitis) to be at 45 cases per million. […] Hypersensitivity vasculitis reportedly has an incidence of 10-30 cases per million people per year. […] Hypersensitivity vasculitis is reported most often in the white population, but epidemiologic studies are not available to assess whether hypersensitivity vasculitis is associated with any specific ethnic group or skin type. […] Although hypersensitivity vasculitis appears to affect men and women in approximately equal numbers, some of the studies from Spain suggest that hypersensitivity vasculitis is slightly more common in men than in women. […] Hypersensitivity vasculitis may occur at any age, but adults are more commonly affected.

• #23 Central nervous system vasculitis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/central-nervous-system-vasculitis-2?lang=us

  Primary angiitis of the CNS remains a rare disorder with an estimated average annual incidence rate of 2.4 cases per million. It affects patients of all ages, but peaks at around 50 years of age, with males affected more commonly than females. […] Secondary causes of CNS vasculitis far exceed the number of cases of primary angiitis of the CNS.

• #24

  https://link.springer.com/article/10.1007/s10157-013-0830-8

  Different vasculitic syndromes present in different age groups. […] Major differences exist worldwide in ANCA specificity which are not entirely related to different phenotypes. […] International studies have given important clues to possible aetiology including silica dust and infection and genetic influences, as shown by the recently published genome-wide association study which revealed that single-nucleotide polymorphisms associate more strongly with ANCA than clinical syndromes. […] Watts RA, Scott DGI. Epidemiology of vasculitis. In: Bridges L, Ball G, editors. Vasculitis. 3rd ed. Oxford: Oxford University Press; 2013 (in press). […] Watts RA, Scott DGI. ANCA vasculitis: to lump or split? Rheumatology. 2012;. doi:10.1093/rheumatology/kes230. […] Watts RA, Gonzlez-Gay M, Garcia-Porrua C, Lane S, Bentham G, Scott DGI. Geoepidemiology of systemic vasculitis. Ann Rheum Dis. 2001;60:1702. […] Fujimoto S, Watts RA, Kobayashi S, Suzuki K, Jayne DRW, Scott GDI, Hashimoto H, Nunoi H. Comparison of the epidemiology of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis between Japan and UK. Rheumatology. 2011;50:191620.

• #25 Systemic vasculitis: epidemiology, classification and environmental factors | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/59/3/161

  The systemic vasculitides continue to present the practising clinician with many challenges. The spectrum and severity of disease is broad from life or sight threatening fulminant disease to relatively minor skin disease. […] There are sufficient differences even within the limited epidemiological data available to suggest that not all these influences work in the same direction in the various described vasculitis syndromes. This editorial will concentrate on recent developments in the classification, epidemiology, and role of environmental factors in pathogenesis. […] The classification of vasculitis remains controversial. Although the American College of Rheumatology (ACR) in 1990 produced classification criteria for the major conditions and the Chapel Hill Consensus Conference (CHCC) in 1994 developed definitions, there are still a number of difficulties.

• #26 Systemic Vasculitis: An Epidemiological Perspective

  https://eurekaselect.com/public/article/33875

  The aetiology of the systemic vasculitides is unknown, clues to the causation may be revealed by their epidemiology. The epidemiology of vasculitis poses considerable challenges to epidemiologists. These challenges include the difficulty of defining a case with a lack of clear distinction between the different disorders, case capture and case ascertainment. The vasculitides are generally rare and therefore a large population is required to accurately determine incidence and prevalence, and this poses questions of feasibility. Nonetheless, despite these difficulties a considerable body of data on the epidemiology of the vasculitides has developed over the past 30 years with interesting age, geographic and ethnic tropisms gradually being revealed. Most of the data comes from Caucasian populations of European descent. In this article we describe the epidemiology of systemic vasculitis focusing on possible risk factors such as age, ethnicity, infection, drugs and other possible environmental agents.

• #27 Clinical Epidemiology and serological/plasma and genetic factors in systemic vasculitis • Johns Hopkins Rheumatology

  https://www.hopkinsrheumatology.org/research-studies/clinical-epidemiology-and-serological-plasma-and-genetic-factors-in-systemic-vasculitis/

  Purpose of Study: This is a longitudinal, observational study of individuals with vasculitis. Our objective is to improve knowledge about the etiology and risk factors for vasculitis. We will also be studying the effects of treatment on vasculitis. […] Dr. Geetha’s clinical interests include renal disease in vasculitis patients with a focus on ANCA associated vasculitis and Henoch-Schonlein Purpura. She does clinical and translational research in vasculitis with a focus on ANCA associated vasculitis and renal disease.

• #28 ANCA Vasculitis – Epidemiology Forecast – 2032

  https://www.researchandmarkets.com/reports/5525942/anca-vasculitis-epidemiology-forecast-2032?srsltid=AfmBOorEMUhg3oAc5b3D2tipOwK9ueQksVpyMABpsPXJvxUkNH62rqdg

  The ANCA Vasculitis epidemiology report gives a thorough understanding of the ANCA Vasculitis by including details such as disease definition, symptoms, causes, pathophysiology, and diagnosis. […] The ANCA Vasculitis epidemiology division provides insights about historical and current patient pool and forecasted trend for every seven major countries. […] The ANCA Vasculitis epidemiology segment covers the epidemiology data in the US, EU5 countries (Germany, Spain, Italy, France, and the UK), and Japan from 2019 to 2032. […] The ANCA Vasculitis report also provides the epidemiology trends observed in the 7MM during the study period, along with the assumptions undertaken. […] The report provides insight into the historical and forecasted patient pool of ANCA Vasculitis in seven major markets covering the United States, EU5 (Germany, Spain, France, Italy, UK), and Japan.

• #29 ANCA Vasculitis – Epidemiology Forecast – 2032

  https://www.researchandmarkets.com/reports/5525942/anca-vasculitis-epidemiology-forecast-2032?srsltid=AfmBOorEMUhg3oAc5b3D2tipOwK9ueQksVpyMABpsPXJvxUkNH62rqdg

  The calculated data are presented with relevant tables and graphs to give a clear view of the epidemiology at first sight. […] The ANCA Vasculitis Epidemiology Report and Model provide an overview of the global trends of ANCA Vasculitis in the seven major markets (7MM: US, France, Germany, Italy, Spain, UK, and Japan). […] The report helps recognize the growth opportunities in the 7MM for the patient population. […] The report assesses the disease risk and burden and highlights the unmet needs of ANCA Vasculitis. […] The ANCA Vasculitis epidemiology covered in the report provides historical as well as forecasted ANCA Vasculitis epidemiology scenario in the 7MM covering the United States, EU5 countries (Germany, Spain, Italy, France, and the United Kingdom), and Japan from 2019 to 2032.

• #30 Global epidemiology of vasculitis

  https://escholarship.org/uc/item/49n2v398

  Global epidemiology of vasculitis […] Accurate estimation of the incidence and prevalence has been hampered by the absence of reliable diagnostic criteria and the rarity of these conditions; however, much progress has been made over the past two decades, although data are still lacking from many parts of the world including the Indian subcontinent, China, Africa and South America. […] Much work needs to be done to better understand the influence of ethnicity, geographical location, environment and social factors on the development of vasculitis.

• #31 Vasculitides – CanVasc

  https://canvasc.ca/vasculitides/

  Vasculitides are rare diseases. There are only few epidemiological studies on vasculitides in adults in Canada, and only a few more have been done in the United States. However, based on European, Australian, New-Zealander and Japanese studies, one can estimate the overall prevalence and annual incidence ranges of primary vasculitides as follows: […] Vasculitis Prevalence (per million) Remarks Annual Incidence (per million) Remarks GCA 500-7,500 (of population 50 y-o) Crude estimates (only few specific studies: 144 in Reunion isle in 2018 with biopsy +; 753 in Sweden with biopsy + in 2015) 80-300 (of population 50 y-o) Down to 5 in Israel in late 1960s; 27 in Reunion isle 2005-2018; up to 370 in Norway in mid 1990s; 20 in subjetcs 60 and up to 520 in those 80 y-o in Minnesota; 79 in Paris in 2017 Takayasu 4 to 8 4.7 to 7 in the UK in early 2000s; Up to 40 in Japan (no epidemiological data for India, but probably at least the same) 1 to 2 Down to 0.4 in Germany; Up to 2.6 in Minnesota in late 1970s, and 3.3 in Kuwait PAN 6 to 30 Up to 30 in late 1990s before HBV-related PAN almost disappeared 0.9-6.8 Up to 8 in the UK, 16 in Kuwait in the late 1990s, and 77 in Alaska in late 1980s (HBV endemy) Kawasaki Acute disease (in general, but damage) 90-500 (of children 5 y-o) Down to 16 in Czech republic in late 1990s; Up to 2,180 in Japan; in US, 91 in Caucasian vs. 320 in Asian children in early 2000s GPA 50-250 Down to 23 in Paris in 2000, 30 in NYC in 1990; Up to 135 in the UK in 2013, 160 in Sweden in 2007, 201 in Germany in 2013-2016, and 260 in Norway in 2013-2015 5 to 30 Down to almost 0 in Japan, 2.9 in Spain; Up to 11 in Australia and Minnesota; 14 in adults in the UK between 2000-2013, 15.6 in Norway (1999-2013), and 34 in Germany in 2013-2016 MPA 25-150 Down to 25 In Paris in 2000; Up to 160 in Norway in 2015 5 to 15 Down to 2.7 In Germany in 2000s;ip to 15 in Japan, and 24 in Kuwait, and 13 in Germany 2013-2016 EGPA 10 to 40 Down to 7 in Germany in 1994; Up to 22 in Australia in 2004, and 42 in Norway in 2015 1 to 2 Down to 0 in Japan; Up to 2.7 in the UK in late 1990s, 2.7 in Norway in 1999-2013, and 4 in Minnesota in late 1970s Behet 10-500 Extremely wide ethnic variations (from 6 in the UK in late 1970s to 4,200 in Turkey in 2000); 100-300 in US , mainly in immigrants; 24 in European-descent vs. 175 in Asian-descent vs 350 in Norh-African- descent French population No precise estimation (chronic disease and wide geographical differences); around 4 in Minnesota in mid 2000s CNS-V No data 2.4 Only 1 study, in Minnesota

• #32 Epidemiology of vasculitis in Europe | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/60/12/1156.2

  We recently compared the annual incidence of primary systemic vasculitis (PSV) in two different regions of Europe (Norwich, UK (latitude 52N) and Lugo, Spain (latitude 43N)). Wegener’s granulomatosis (WG) was more common in Norwich (10.6/million) than in Spain (4.9/million), though the overall incidence of PSV was similar. This supports the idea that environmental factors may be important in the aetiopathogenesis of PSV. To extend our observations we have now studied the incidence of PSV in northern Europe (Troms, Norway (latitude 70N)).

• #33 Vasculitis (Causes, Symptoms, and Treatment)

  https://patient.info/doctor/vasculitis-pro

  Vasculitis is rare. About 14,000 new cases are diagnosed in the UK each year. A UK general practice study found that the most common type – polymyalgia rheumatica (PR) – had an estimated cumulative prevalence (the number of people who had ever had the disease over a given period of time) of 2.27%. The corresponding figure for the next most common – giant cell arteritis (GCA) – was 0.41%. […] The prognosis of ANCA-associated vasculitis has been transformed in recent years. Once it was a set of invariably acute and fatal conditions, but these disorders are currently considered to be chronic diseases. This change is largely attributable to earlier diagnosis and the use of immunotherapeutics. However, patients still experience premature mortality, relapse, comorbid ill health and poor quality of life.

• #34 Arterial and venous thrombosis in systemic and monogenic vasculitis | Nature Reviews Rheumatology

  https://www.nature.com/articles/s41584-025-01252-7

  Systemic vasculitis, common forms of which include anti-neutrophil cytoplasmic antibody-associated small-vessel vasculitis, large-vessel vasculitis and Behet syndrome, are frequently complicated by arterial or venous thrombotic events (AVTEs). […] Newly identified entities such as DADA2 (deficiency of adenosine deaminase 2) and VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome, which are driven by genetic mutations, also exhibit vasculitic features and are associated with a high risk of AVTEs. […] AVTEs in systemic vasculitis, including monogenic forms of vasculitis, are due to the complex interaction of inflammation and coagulation. […] New insights into the pathogenetic mechanisms implicate endothelial dysfunction, immune complex deposition and the interplay of pro-inflammatory cytokines with prothrombotic factors, which collectively promote thrombus formation.

• #35 Arterial and venous thrombosis in systemic and monogenic vasculitis | Nature Reviews Rheumatology

  https://www.nature.com/articles/s41584-025-01252-7

  AVTEs impose a substantial disease burden, complicate diagnosis and negatively affect prognosis by increasing the risk of morbidity and mortality. […] Early diagnosis and treatment are crucial to prevent lasting damage. […] Management strategies should target both thrombosis and underlying inflammation. […] Antithrombotic therapies, including low-dose aspirin, or oral anticoagulants should be used on the basis of individual thrombotic risk assessment. […] Immunosuppressive therapy is the cornerstone of treatment for arterial and venous thrombosis, particularly in Behet syndrome, in which vascular inflammation has a crucial role in thrombotic complications. […] Systemic vasculitis, including anti-neutrophil cytoplasmic antibody-associated vasculitis, large-vessel vasculitis and Behet syndrome, is frequently complicated by arterial or venous thrombotic events.

• #36 ANCA Associated Vasculitis Industry & Epidemiology Analysis

  https://www.globenewswire.com/news-release/2023/07/21/2708731/0/en/ANCA-Associated-Vasculitis-Industry-Epidemiology-Analysis-and-Forecast-2032-Includes-Insights-Into-Drugs-and-Key-Players.html

  The total market size of ANCA Associated Vasculitis in the 7MM is approximately USD 1,220 million in 2022 and is projected to increase during the forecast period (2023-2032). […] The market size in the 7MM will increase at a CAGR of 6.9% due to increasing awareness of the disease and the launch of the emerging therapy.

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