Materiały źródłowe

• #1 Behcet Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470257/

  Behcet disease is an auto-inflammatory systemic vasculitis of unknown etiology. […] The exact etiological basis of Behcet disease remains unknown, although genetics and environmental factors have been found to play a role. […] Increased prevalence along the „Silk Route” and familial aggregation suggest a genetic element, although Behcet disease does not follow a mendelian inheritance. […] The most frequent association is with carriers of HLA-B51/B5, who are at high risk for developing Behcet disease compared to non-carriers. […] Several other genes have been identified, including tumor necrosis factor (TNF), heat shock proteins, and major histocompatibility complex class I chain-related genes. […] Exposure to infectious agents, especially hypersensitivity to Streptococcus sanguinis antigens, has suggested a pathological role.

• #2 Potential Infectious Etiology of Behçet’s Disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3255303/

  Behet’s disease is a multisystem inflammatory disorder characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. The cause of Behet’s disease remains unknown, but epidemiologic findings suggest that an autoimmune process is triggered by an environmental agent in a genetically predisposed individual. […] An infectious agent could operate through molecular mimicry, and subsequently the disease could be perpetuated by an abnormal immune response to an autoantigen in the absence of ongoing infection. […] The cause of Behet’s disease remains unknown, but epidemiologic findings suggest that an autoimmune process is triggered by an infectious or environmental agent (possibly local to a geographic region) in a genetically predisposed individual. […] As is the case of other autoimmune diseases, there is interest in an infectious etiology. Although there is no information supporting the role of a single microorganism as the specific cause, a problem with dysregulation in innate immunity, with an altered response to more than one infectious agent, is a generally accepted theory.

• #3 Behçet’s Disease

  https://rheumatology.org/patients/behcets-disease

  Behets (beh-CHETS) disease is a rare illness that affects the bodys blood vessels. […] We dont know exactly what causes Behets disease, but there are certain genes that seem to be involved. […] People from the Middle East, East Asia (China, Japan), Turkey, and Iran are more likely to develop this condition.

• #4 Behcet disease | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/behcet-disease

  Behcet disease might be an autoimmune disorder, which means the body’s immune system attacks some of its own healthy cells by mistake. It’s likely that gene changes and factors in the environment play a role. […] Experts believe that swelling of the blood vessels, called vasculitis, causes the symptoms of Behcet disease. The condition can involve arteries and veins of all sizes. Vasculitis can damage blood vessels all through the body. […] Some genes might make people more likely to get Behcet disease. Some researchers think that a germ can trigger the disease in people who have these genes.

• #5 Behcet Disease: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/329099-overview

  Behet disease is a rare vasculitic disorder that is characterized by recurrent oral aphthous ulcers, genital ulcers, and uveitis. […] The disease appears to involve an autoimmune response triggered by exposure to an infectious agent, and it occurs predominantly in people with ancestors along the Silk Road, the ancient route that connected Asia with the Middle East and southern Europe. […] Theories behind the pathogenesis of Behet disease currently suggest an autoimmune etiology. It is thought that in genetically predisposed individuals, exposure to an infectious agent or an environmental antigen triggers the autoimmune response. […] The specific etiology of Behet disease remains elusive, but the interplay between infectious-agent exposure and genetic factors may have a role. An environmentally triggered hyperactive primed state of autoimmunity ensues, resulting in two types of vascular damage. The first is vasculitic lesions that may be widespread. Sequelae depend on the various organ systems affected.

• #6

  https://www.nhs.uk/conditions/behcets-disease/

  The cause of Behet’s disease is unknown, although most experts believe it’s an autoinflammatory condition. […] In Behet’s disease, this involves inflammation of the blood vessels. This is thought to be caused by a problem with the immune system. […] It’s not clear what triggers this problem with the immune system, but 2 things are thought to play a role: genes Behet’s disease tends to be much more common in certain ethnic groups where the genes that are linked to the condition may be more common […] environmental factors although a specific environmental factor has not been identified, rates of Behet’s disease are lower in people from an at-risk ethnic group who live outside their native country.

• #7 Behçet’s disease – Wikipedia

  https://en.wikipedia.org/wiki/Beh%C3%A7et%27s_disease

  The cause is unknown. […] It is believed to be partly genetic. […] The primary cause is not well known. In fact, no one knows yet why the immune system starts to behave this way in Behet’s disease. There does however seem to be a genetic component involved, as first degree relatives of the affected patients are often affected in more than the expected proportion for the general population. […] Research suggests that previous infections may provoke the autoimmune responses present in Behet’s disease. […] The primary mechanism of the damage is autoimmune, which by definition is an overactive immune system that targets the patient’s own body. […] An association with the GIMAP („GTPase of the immunity-associated protein”) family of genes on the long arm of chromosome 7 (7q36.1) has been reported.

• #8 Neurological involvement by Behçet’s syndrome: clinical features, diagnosis, treatment and outcome | Practical Neurology

  https://pn.bmj.com/content/23/5/386

  Neurological involvement in Behets syndrome arises predominately through an inflammatory meningoencephalitis characterised by perivenular inflammation due to activation of Th-17 immunological pathways. […] Behets syndrome is an autoinflammatory disorder of unknown cause in which an inflammatory perivasculitis arises within the mucous membranes of the oropharynx, gastrointestinal tract and genital regions. Inflammation of veins leads to thrombosis and inflammation of arteries leads to vasculitis and aneurysm occurs. […] The cardinal feature of Behets disease is an activation of neutrophils, associated with enhancement of helper Th17 immune responses leading to chemokine release and activation of NKT and T cell and Th-17 cell responses. […] Possession of the human leucocyte antigen HLA B-51 confers an OR of 5.90 for developing the disease and there is a correlation between the prevalence of B-51 in the general population and the incidence of the disease. […] Changes to the microbiome of the oropharynx and gut with colonisation of streptococcus and other bacteria may exert a stimulating role on activation of the disease.

• #9 Behcet Disease: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/329099-overview

  Some of the pathologic changes are not vasculitis but due to thrombosis and/or clot formation caused by the development of a hypercoagulable state. The mechanism is still undetermined; however, studies have demonstrated excessive thrombin formation and the potential role of impaired fibrinolytic kinetics in the generation of the hypercoagulable/prothrombotic state. Pathologic activation of the procoagulant cascade via endothelial injury has also been demonstrated in patients with Behet disease.

• #10 Behcet Disease: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/329099-overview

  Some of the pathologic changes are not vasculitis but due to thrombosis and/or clot formation caused by the development of a hypercoagulable state. The mechanism is still undetermined; however, studies have demonstrated excessive thrombin formation and the potential role of impaired fibrinolytic kinetics in the generation of the hypercoagulable/prothrombotic state. Pathologic activation of the procoagulant cascade via endothelial injury has also been demonstrated in patients with Behet disease.

• #11 Behcet Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470257/

  Behcet disease is an auto-inflammatory systemic vasculitis of unknown etiology. […] The exact etiological basis of Behcet disease remains unknown, although genetics and environmental factors have been found to play a role. […] Increased prevalence along the „Silk Route” and familial aggregation suggest a genetic element, although Behcet disease does not follow a mendelian inheritance. […] The most frequent association is with carriers of HLA-B51/B5, who are at high risk for developing Behcet disease compared to non-carriers. […] Several other genes have been identified, including tumor necrosis factor (TNF), heat shock proteins, and major histocompatibility complex class I chain-related genes. […] Exposure to infectious agents, especially hypersensitivity to Streptococcus sanguinis antigens, has suggested a pathological role.

• #12 Behcet’s Disease : Johns Hopkins Vasculitis Center

  https://www.hopkinsvasculitis.org/types-vasculitis/behcets-disease/

  What causes Behcets Disease? […] Behcets is one of the few forms of vasculitis in which there is a known genetic predisposition. The presence of the gene HLAB51 is a risk factor for this disease. However, it must be emphasized that presence of the gene in and of itself is not enough to cause Behcets: many people possess the gene, but relatively few develop Behcets. Despite the predisposition to Behcets conferred by HLAB51, familial cases are not the rule, constituting only about 5% of cases. Thus, it is believed that other factors (perhaps more than one) play a role. Possibilities include infections and other environmental exposures.

• #13 Behçet disease: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/behcet-disease/

  Behçet disease is an inflammatory condition that affects many parts of the body. […] The cause of Behçet disease is unknown. The condition probably results from a combination of genetic and environmental factors, most of which have not been identified. […] However, a particular variation in the HLA-B gene has been associated with the risk of developing Behçet disease. […] Other genetic and environmental factors likely contribute to the risk of Behçet disease. Researchers are studying several genes related to immune system function. It also appears likely that environmental factors, such as certain bacterial or viral infections, play a role in triggering the disease in people who are at risk. However, the influence of genetic and environmental factors on the development of this complex disorder remains unclear.

• #14 Behçet’s Disease (Causes, Symptoms, and Treatment)

  https://patient.info/doctor/behcets-disease-pro

  Behet’s disease is a complex multi-system disorder of unknown aetiology characteristically presenting with recurrent oral ulcers. […] Although the pathogenesis of Behet’s disease is unclear, some studies have shown that immunological aberrations play an important role in the development and progression of Behet’s disease. […] Infection-related trigger factors, including antigens and autoantigens, are believed to mediate the development of Behet’s disease in patients with a genetic predisposition, resulting in the production of numerous cytokines and chemokines to combat the infection-related factors. […] HLA-B51 is the most strongly associated known genetic factor. However, it accounts for less than 20% of the genetic risk, even in familial cases (less than 5%), which indicates that other genetic factors remain to be discovered.

• #15 Behcet Disease – EyeWiki

  https://eyewiki.org/Behcet_Disease

  Behcets disease (BD) is a multisystem vasculitis of unknown etiology. […] Behets disease still remains a disease of unknown etiology. […] Given that HLA-B51 (particularly HLA-B5101) has been associated with BD, it is proposed that a genetic predisposition exists, but the actual association has not been well defined. […] There are reports of familial clustering, but it is actually rare to see other family members affected with BD, even when many family members have the HLA-B51 gene. This fact makes the presence of HLA-B51 alone a minor factor. […] Given that BD is of unknown etiology, the highest prevalence of the disease is found in the Middle East and Far East, particularly Japan. Since the same clinical manifestations are found across different ethnic groups, it is presumed that environmental factors likely also play a role along with genetic predisposition.

• #16 Behcet Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470257/

  Behcet disease is an auto-inflammatory systemic vasculitis of unknown etiology. […] The exact etiological basis of Behcet disease remains unknown, although genetics and environmental factors have been found to play a role. […] Increased prevalence along the „Silk Route” and familial aggregation suggest a genetic element, although Behcet disease does not follow a mendelian inheritance. […] The most frequent association is with carriers of HLA-B51/B5, who are at high risk for developing Behcet disease compared to non-carriers. […] Several other genes have been identified, including tumor necrosis factor (TNF), heat shock proteins, and major histocompatibility complex class I chain-related genes. […] Exposure to infectious agents, especially hypersensitivity to Streptococcus sanguinis antigens, has suggested a pathological role.

• #17 Behçet’s disease – Wikipedia

  https://en.wikipedia.org/wiki/Beh%C3%A7et%27s_disease

  The cause is unknown. […] It is believed to be partly genetic. […] The primary cause is not well known. In fact, no one knows yet why the immune system starts to behave this way in Behet’s disease. There does however seem to be a genetic component involved, as first degree relatives of the affected patients are often affected in more than the expected proportion for the general population. […] Research suggests that previous infections may provoke the autoimmune responses present in Behet’s disease. […] The primary mechanism of the damage is autoimmune, which by definition is an overactive immune system that targets the patient’s own body. […] An association with the GIMAP („GTPase of the immunity-associated protein”) family of genes on the long arm of chromosome 7 (7q36.1) has been reported.

• #18 Behcet Syndrome: Background, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/1006358-overview

  Behet syndrome is a multisystem disease of unknown etiology probably first described by Hippocrates in the 5th century. […] Although the cause of the disease is still unknown, it has become recognized as a multisystemic inflammatory disease with a heterogeneity of clinical manifestations. […] Results of recent genome-wide association studies (GWASs) confirm the association with HLA-B51, although there is currently not a diagnostic or prognostic role for the presence of HLA-B51. […] These GWASs demonstrate that the most common nonHLA association is with the interleukin (IL)10 and IL23R loci and underline the essential role of the IL-10 and IL23/17 pathways in the pathogenesis of Behet syndrome. […] An increased incidence of skin pathergy and HLA-B51 antigen is observed in Middle Eastern and Asian patients, compared with North American or northern European patients.

• #19 Behcet Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470257/

  Behcet disease is an auto-inflammatory systemic vasculitis of unknown etiology. […] The exact etiological basis of Behcet disease remains unknown, although genetics and environmental factors have been found to play a role. […] Increased prevalence along the „Silk Route” and familial aggregation suggest a genetic element, although Behcet disease does not follow a mendelian inheritance. […] The most frequent association is with carriers of HLA-B51/B5, who are at high risk for developing Behcet disease compared to non-carriers. […] Several other genes have been identified, including tumor necrosis factor (TNF), heat shock proteins, and major histocompatibility complex class I chain-related genes. […] Exposure to infectious agents, especially hypersensitivity to Streptococcus sanguinis antigens, has suggested a pathological role.

• #20 Behcet Disease – EyeWiki

  https://eyewiki.org/Behcet_Disease

  Behcets disease (BD) is a multisystem vasculitis of unknown etiology. […] Behets disease still remains a disease of unknown etiology. […] Given that HLA-B51 (particularly HLA-B5101) has been associated with BD, it is proposed that a genetic predisposition exists, but the actual association has not been well defined. […] There are reports of familial clustering, but it is actually rare to see other family members affected with BD, even when many family members have the HLA-B51 gene. This fact makes the presence of HLA-B51 alone a minor factor. […] Given that BD is of unknown etiology, the highest prevalence of the disease is found in the Middle East and Far East, particularly Japan. Since the same clinical manifestations are found across different ethnic groups, it is presumed that environmental factors likely also play a role along with genetic predisposition.

• #21 What is Behcets Disease

  https://behcetdiseasesociety.org/menu/25/what-is-behcets-disease

  Behcet`s disease is a systemic vasculitis (inflammation of large and small blood vessels) of an unknown cause. […] Scientists do not know the exact cause of Behcet`s disease, but extensive work is ongoing. Most symptoms are caused by inflammation of blood vessels. […] Doctors think that an autoimmune reaction may cause the blood vessels to become inflamed, but they do not know what triggers this reaction. […] First, it is believed that abnormalities of the immune system make some people susceptible to the disease, scientists believe that this susceptibility may be inherited; that is, it may be due to one or more specific genes (but this does not mean that it is transmitted like inherited disease). […] Second, something in the environment, possible a bacterium or virus, might trigger or activate the disease in susceptible people.

• #22 Behçet’s disease: Treatment, lifestyle tips, and causes

  https://www.medicalnewstoday.com/articles/310313

  The exact cause of Behets disease is unknown, but it is thought to be an autoimmune disease. […] Certain groups of people have a higher risk of developing the disease than others. […] Age: All ages and sexes are at risk of developing Behets disease, but it most commonly affects people in their 20s and 30s. Men typically experience more severe symptoms than women. […] Ethnicity and geographic location: These may play a role in how likely a person is to develop Behets disease. The condition is most common in men from the Middle East and Asia, and women from the United States, other Western Countries, Japan, and Korea. […] Genetic and hereditary factors: There may be a genetic or inherited component to the disease. Behets disease could also be linked to bacteria, viruses, or environmental factors. More research is needed, however, before these suggestions can be confirmed.

• #23 Potential Infectious Etiology of Behçet’s Disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3255303/

  A viral cause was first postulated by Behet in 1937. Evidence of ongoing infection with a variety of viral agents has been sought. However, often there is only a history of previous infection and/or seropositivity. […] Several other viral agents, including hepatitis C virus, parvovirus B19, cytomegalovirus, Epstein-Barr virus, and varicella zoster virus, may also have some role. […] Potential bacteria are a variety of streptococcal antigens, Saccharomyces cerevisiae, mycobacteria, Borrelia burgdorferi, Helicobacter pylori, Escherichia coli, Staphylococcus aureus, Mycoplasma fermentans. […] The infectious model is also supported by observations that oral ulcers precede the establishment of disease by months or years and disease relapses are frequent. […] The relationship between streptococcal infections and Behet’s disease is suggested by clinical observations that an unhygienic oral condition including periodontitis, decayed teeth, and chronic tonsillitis is frequently noted in the oral cavity of Bechet’s disease patients.

• #24 Potential Infectious Etiology of Behçet’s Disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3255303/

  It is not clear that the predisposition of the patients is correlated with streptococcal infection, but the uncommon oral Streptococcus sanguinis serotypes (called KTH-1) and antibodies against the bacteria are significantly increased in the oral flora and serum, respectively, of patients with the disease compared with healthy controls. […] The new criteria included hypersensitivity skin reactions against streptococci in the diagnosis as one of the references and the levels of disease severity of Behet’s disease patients. […] Thus, HSPs are possible candidate antigen for Behet’s disease. […] Studies have shown that S. sanguis and HSP 60/65kDa activate T cells in Behet’s disease patients but not controls. […] Pustular skin lesions are often not sterile and may contain Staphylococcus aureus and Prevotella species.

• #25 Behcet Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470257/

  While many other infectious agents, including Staphylococcus aureus, Herpes simplex virus type 1, and Prevotella species, have been suggested as potential culprits, their direct association with the development of Behcet disease has not been confirmed. […] The current belief is that exposure to an infectious or an external agent somehow initiates an auto-inflammatory response in genetically predisposed individuals.

• #26 Potential Infectious Etiology of Behçet’s Disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3255303/

  A viral cause was first postulated by Behet in 1937. Evidence of ongoing infection with a variety of viral agents has been sought. However, often there is only a history of previous infection and/or seropositivity. […] Several other viral agents, including hepatitis C virus, parvovirus B19, cytomegalovirus, Epstein-Barr virus, and varicella zoster virus, may also have some role. […] Potential bacteria are a variety of streptococcal antigens, Saccharomyces cerevisiae, mycobacteria, Borrelia burgdorferi, Helicobacter pylori, Escherichia coli, Staphylococcus aureus, Mycoplasma fermentans. […] The infectious model is also supported by observations that oral ulcers precede the establishment of disease by months or years and disease relapses are frequent. […] The relationship between streptococcal infections and Behet’s disease is suggested by clinical observations that an unhygienic oral condition including periodontitis, decayed teeth, and chronic tonsillitis is frequently noted in the oral cavity of Bechet’s disease patients.

• #27 Behçet’s disease in children, an overview | Pediatric Rheumatology | Full Text

  https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-016-0070-z

  The aetiology of BD is still unknown; however, it appears to be a complex disease relying on an interaction between the genetic background and the environment. […] The underlying mechanisms causing the disease are still unclear and could combine both autoinflammation and autoimmunity. […] The recent discovery of A20 protein haploinsufficiency as a cause of autosomal dominant Behet-like disease strongly supports the idea that the full spectrum of disorders meeting criteria for BD cannot be related to a single mechanism. […] Pathogens such as herpes simplex virus, streptococci, staphylococci, or Escherichia species, and heat-shock proteins may contribute to attacks of BD in susceptible persons via stimulation of the innate immune system (inflammasome activation) through TLR2 and TLR4. […] Improvement of dental and periodontal hygiene has been associated with a decrease of oral ulcerations, and could be one factor explaining the decrease of the incidence of BD in countries like Japan and Turkey.

• #28 Potential Infectious Etiology of Behçet’s Disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3255303/

  It is not clear that the predisposition of the patients is correlated with streptococcal infection, but the uncommon oral Streptococcus sanguinis serotypes (called KTH-1) and antibodies against the bacteria are significantly increased in the oral flora and serum, respectively, of patients with the disease compared with healthy controls. […] The new criteria included hypersensitivity skin reactions against streptococci in the diagnosis as one of the references and the levels of disease severity of Behet’s disease patients. […] Thus, HSPs are possible candidate antigen for Behet’s disease. […] Studies have shown that S. sanguis and HSP 60/65kDa activate T cells in Behet’s disease patients but not controls. […] Pustular skin lesions are often not sterile and may contain Staphylococcus aureus and Prevotella species.

• #29 Potential Infectious Etiology of Behçet’s Disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3255303/

  The role of microorganisms in the pathogenesis of Behet’s disease has long been investigated, and there are ample data on several microorganisms. However, none of these infectious agents have been proved to cause Behet’s disease. Thus, a complex immune response might be generated to a group of microorganisms that share common antigens rather than to an infection due to a specific single microorganism.

• #30

  https://journals.lww.com/neur/fulltext/2018/66060/focus_on_neuro_beh_et_s_disease__a_review.17.aspx

  In summary, the epidemiologic findings suggest that the autoimmune process is triggered by an infectious or environmental agent specific for the geographic region. […] Moreover, there is still no information supporting the hypothetical role of a single microorganism as a specific cause of autoimmune dysregulation.

• #31 Behçet’s Disease: Symptoms, Treatments, Causes & Diagnosis

  https://my.clevelandclinic.org/health/diseases/12980-behcets-disease

  Behet’s disease, also known as Silk Road disease, is a chronic (long-term) inflammatory condition, caused by vasculitis (blood vessel inflammation), which will affect and damage both your arteries and veins. […] Scientists are still not exactly sure what causes this disease. It may be an autoimmune disorder, meaning the body’s immune system mistakenly attacks healthy tissue with an inflammatory response. Both HLA-B5 and HLA-B51 are gene markers that are sometimes present in patients with Behets disease. But there are some people who have this gene marker who don’t have Behets disease. Researchers are studying other genes related to immune function and think that infections (either bacteria or a virus) may play a role in triggering the disease in some people who have genetic markers that predispose them to Behets. […] Genetic and environmental factors are both likely factors in the development of Behets disease.

• #32 Behçet Disease – Bone, Joint, and Muscle Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/bone-joint-and-muscle-disorders/vasculitic-disorders/beh%C3%A7et-disease

  Behet disease occurs worldwide but is most common in the area along the silk route from the Mediterranean to China. It is relatively uncommon in the United States. […] The cause of Behet disease is unknown. The risk appears to be higher in people who have certain genetic characteristics, such as people who have a gene called HLA-B51.

• #33 Behçet’s Syndrome – Vasculitis Foundation

  https://vasculitisfoundation.org/education/vasculitis-types/behcets-syndrome/

  Behets syndrome is a form of vasculitis, a family of rare disorders characterized by inflammation of the blood vessels, which can restrict blood flow and damage vital organs and tissues. […] The cause of Behets is not fully understood. Vasculitis is classified as an autoimmune disorder, a disease that occurs when the body’s natural defense system mistakenly attacks healthy tissue. Behets is one of the few forms of vasculitis where a specific gene HLA-B51 is a known risk factor for the syndrome. However, this gene is also seen in the general population, and not everyone who has it gets Behets. Therefore, it is believed that an infection and/or environmental factors may play a role in the onset of this disease. […] Behets mainly affects people in their 20s and 30s, but the syndrome can occur in individuals of all ages. While Behets occurs in both men and women, the disease is usually more severe in males. […] People from the Mediterranean, the Middle East and Asia are more likely to get this disease; it is rare in the US. Prevalence is estimated at 3 to 5 per 100,000 people in the US. Turkey has the highest prevalence, with approximately 400 cases per 100,000 people.

• #34 Behçet’s disease: Treatment, lifestyle tips, and causes

  https://www.medicalnewstoday.com/articles/310313

  The exact cause of Behets disease is unknown, but it is thought to be an autoimmune disease. […] Certain groups of people have a higher risk of developing the disease than others. […] Age: All ages and sexes are at risk of developing Behets disease, but it most commonly affects people in their 20s and 30s. Men typically experience more severe symptoms than women. […] Ethnicity and geographic location: These may play a role in how likely a person is to develop Behets disease. The condition is most common in men from the Middle East and Asia, and women from the United States, other Western Countries, Japan, and Korea. […] Genetic and hereditary factors: There may be a genetic or inherited component to the disease. Behets disease could also be linked to bacteria, viruses, or environmental factors. More research is needed, however, before these suggestions can be confirmed.

• #35 Behçet’s disease | nidirect

  https://www.nidirect.gov.uk/conditions/behcets-disease

  The cause of Behet’s disease is unknown. Although most experts believe it’s an autoinflammatory condition. […] In cases of Behet’s disease, it’s thought the immune system mistakenly attacks the blood vessels. […] It’s not clear what triggers this problem with the immune system. But two things are thought to play a role: genes and environmental factors. […] Behet’s disease tends to be much more common in certain ethnic groups, where the genes that are linked to the condition may be more common. […] Although a specific environmental factor hasn’t been identified, rates of Behet’s disease are lower in people from an at-risk ethnic group, who live outside their native country. […] Behet’s disease is more common in the Far East, the Middle East and Mediterranean countries such as Turkey, Iran and Israel. […] People of Mediterranean, Middle Eastern and Asian origin are thought to be most likely to develop the condition. Although the condition can affect all ethnic groups.

• #36 Behçet’s Syndrome – Vasculitis Foundation

  https://vasculitisfoundation.org/education/vasculitis-types/behcets-syndrome/

  Behets syndrome is a form of vasculitis, a family of rare disorders characterized by inflammation of the blood vessels, which can restrict blood flow and damage vital organs and tissues. […] The cause of Behets is not fully understood. Vasculitis is classified as an autoimmune disorder, a disease that occurs when the body’s natural defense system mistakenly attacks healthy tissue. Behets is one of the few forms of vasculitis where a specific gene HLA-B51 is a known risk factor for the syndrome. However, this gene is also seen in the general population, and not everyone who has it gets Behets. Therefore, it is believed that an infection and/or environmental factors may play a role in the onset of this disease. […] Behets mainly affects people in their 20s and 30s, but the syndrome can occur in individuals of all ages. While Behets occurs in both men and women, the disease is usually more severe in males. […] People from the Mediterranean, the Middle East and Asia are more likely to get this disease; it is rare in the US. Prevalence is estimated at 3 to 5 per 100,000 people in the US. Turkey has the highest prevalence, with approximately 400 cases per 100,000 people.

• #37 Behcet’s Disease | Behcet’s Syndrome | MedlinePlus

  https://medlineplus.gov/behcetssyndrome.html

  Doctors aren’t sure what causes Behcet’s. […] It is rare in the United States, but is common in the Middle East and Asia.

• #38

  https://www.nhs.uk/conditions/behcets-disease/

  The cause of Behet’s disease is unknown, although most experts believe it’s an autoinflammatory condition. […] In Behet’s disease, this involves inflammation of the blood vessels. This is thought to be caused by a problem with the immune system. […] It’s not clear what triggers this problem with the immune system, but 2 things are thought to play a role: genes Behet’s disease tends to be much more common in certain ethnic groups where the genes that are linked to the condition may be more common […] environmental factors although a specific environmental factor has not been identified, rates of Behet’s disease are lower in people from an at-risk ethnic group who live outside their native country.

• #39 Behçet’s disease | nidirect

  https://www.nidirect.gov.uk/conditions/behcets-disease

  The cause of Behet’s disease is unknown. Although most experts believe it’s an autoinflammatory condition. […] In cases of Behet’s disease, it’s thought the immune system mistakenly attacks the blood vessels. […] It’s not clear what triggers this problem with the immune system. But two things are thought to play a role: genes and environmental factors. […] Behet’s disease tends to be much more common in certain ethnic groups, where the genes that are linked to the condition may be more common. […] Although a specific environmental factor hasn’t been identified, rates of Behet’s disease are lower in people from an at-risk ethnic group, who live outside their native country. […] Behet’s disease is more common in the Far East, the Middle East and Mediterranean countries such as Turkey, Iran and Israel. […] People of Mediterranean, Middle Eastern and Asian origin are thought to be most likely to develop the condition. Although the condition can affect all ethnic groups.

• #40 Behçet’s disease: Treatment, lifestyle tips, and causes

  https://www.medicalnewstoday.com/articles/310313

  The exact cause of Behets disease is unknown, but it is thought to be an autoimmune disease. […] Certain groups of people have a higher risk of developing the disease than others. […] Age: All ages and sexes are at risk of developing Behets disease, but it most commonly affects people in their 20s and 30s. Men typically experience more severe symptoms than women. […] Ethnicity and geographic location: These may play a role in how likely a person is to develop Behets disease. The condition is most common in men from the Middle East and Asia, and women from the United States, other Western Countries, Japan, and Korea. […] Genetic and hereditary factors: There may be a genetic or inherited component to the disease. Behets disease could also be linked to bacteria, viruses, or environmental factors. More research is needed, however, before these suggestions can be confirmed.

• #41 Behcets Disease General Information Research Page

  https://behcets.info/

  This marked geographic variation of BD can be explained by the genetic basis of the disease and/or environmental triggers. […] Male sex and a younger age of onset are associated with more severe disease. […] The clinical severity of the disease showed a significant increase in noncompliant treatment group compared with compliant group with the passage of time. […] The frequency of ocular involvement and GUs was significantly higher in patients whose disease onset was less than 40 years. […] Our study showed that mucocutaneous lesions are the hallmarks of the disease, and especially OUs precede other manifestations.

• #42 Behçet’s Syndrome – Vasculitis Foundation

  https://vasculitisfoundation.org/education/vasculitis-types/behcets-syndrome/

  Behets syndrome is a form of vasculitis, a family of rare disorders characterized by inflammation of the blood vessels, which can restrict blood flow and damage vital organs and tissues. […] The cause of Behets is not fully understood. Vasculitis is classified as an autoimmune disorder, a disease that occurs when the body’s natural defense system mistakenly attacks healthy tissue. Behets is one of the few forms of vasculitis where a specific gene HLA-B51 is a known risk factor for the syndrome. However, this gene is also seen in the general population, and not everyone who has it gets Behets. Therefore, it is believed that an infection and/or environmental factors may play a role in the onset of this disease. […] Behets mainly affects people in their 20s and 30s, but the syndrome can occur in individuals of all ages. While Behets occurs in both men and women, the disease is usually more severe in males. […] People from the Mediterranean, the Middle East and Asia are more likely to get this disease; it is rare in the US. Prevalence is estimated at 3 to 5 per 100,000 people in the US. Turkey has the highest prevalence, with approximately 400 cases per 100,000 people.

• #43 Behcet Disease: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/329099-overview

  Behet disease is a rare vasculitic disorder that is characterized by recurrent oral aphthous ulcers, genital ulcers, and uveitis. […] The disease appears to involve an autoimmune response triggered by exposure to an infectious agent, and it occurs predominantly in people with ancestors along the Silk Road, the ancient route that connected Asia with the Middle East and southern Europe. […] Theories behind the pathogenesis of Behet disease currently suggest an autoimmune etiology. It is thought that in genetically predisposed individuals, exposure to an infectious agent or an environmental antigen triggers the autoimmune response. […] The specific etiology of Behet disease remains elusive, but the interplay between infectious-agent exposure and genetic factors may have a role. An environmentally triggered hyperactive primed state of autoimmunity ensues, resulting in two types of vascular damage. The first is vasculitic lesions that may be widespread. Sequelae depend on the various organ systems affected.

• #44 Learn About Behcet’s Disease Symptoms & Manifestations, Causes, and Management | Behcet’s Connection – Celgene Corporation

  https://www.behcetsconnection.com/about-behcets

  No single underlying cause of Behet’s Disease has been identified. It is thought that genetic predisposition and environmental triggers may work together to activate an autoimmune cascade that leads to Behet’s Disease. The exact mechanism of the inflammatory changes in Behet’s Disease remains unknown. […] Environmental Triggers […] Viral or bacterial agents such as Streptococcus sanguinis, herpes simplex virus, Epstein-Barr virus, and cytomegalovirus […] Genetic Predisposition […] Family history […] HLA-B51 […] IL-10 […] IL23R/IL12RB2 […] Immune Response Activation […] Increased Production Of Pro-inflammatory Cytokines […] IL-2, IL-6, IL-12, IL-17, IL-23, IL-33, TNF-, IFN-

• #45 Potential Infectious Etiology of Behçet’s Disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3255303/

  Behet’s disease is a multisystem inflammatory disorder characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. The cause of Behet’s disease remains unknown, but epidemiologic findings suggest that an autoimmune process is triggered by an environmental agent in a genetically predisposed individual. […] An infectious agent could operate through molecular mimicry, and subsequently the disease could be perpetuated by an abnormal immune response to an autoantigen in the absence of ongoing infection. […] The cause of Behet’s disease remains unknown, but epidemiologic findings suggest that an autoimmune process is triggered by an infectious or environmental agent (possibly local to a geographic region) in a genetically predisposed individual. […] As is the case of other autoimmune diseases, there is interest in an infectious etiology. Although there is no information supporting the role of a single microorganism as the specific cause, a problem with dysregulation in innate immunity, with an altered response to more than one infectious agent, is a generally accepted theory.

• #46 Behçet’s disease in children, an overview | Pediatric Rheumatology | Full Text

  https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-016-0070-z

  The aetiology of BD is still unknown; however, it appears to be a complex disease relying on an interaction between the genetic background and the environment. […] The underlying mechanisms causing the disease are still unclear and could combine both autoinflammation and autoimmunity. […] The recent discovery of A20 protein haploinsufficiency as a cause of autosomal dominant Behet-like disease strongly supports the idea that the full spectrum of disorders meeting criteria for BD cannot be related to a single mechanism. […] Pathogens such as herpes simplex virus, streptococci, staphylococci, or Escherichia species, and heat-shock proteins may contribute to attacks of BD in susceptible persons via stimulation of the innate immune system (inflammasome activation) through TLR2 and TLR4. […] Improvement of dental and periodontal hygiene has been associated with a decrease of oral ulcerations, and could be one factor explaining the decrease of the incidence of BD in countries like Japan and Turkey.

• #47 Learn About Behcet’s Disease Symptoms & Manifestations, Causes, and Management | Behcet’s Connection – Celgene Corporation

  https://www.behcetsconnection.com/about-behcets

  No single underlying cause of Behet’s Disease has been identified. It is thought that genetic predisposition and environmental triggers may work together to activate an autoimmune cascade that leads to Behet’s Disease. The exact mechanism of the inflammatory changes in Behet’s Disease remains unknown. […] Environmental Triggers […] Viral or bacterial agents such as Streptococcus sanguinis, herpes simplex virus, Epstein-Barr virus, and cytomegalovirus […] Genetic Predisposition […] Family history […] HLA-B51 […] IL-10 […] IL23R/IL12RB2 […] Immune Response Activation […] Increased Production Of Pro-inflammatory Cytokines […] IL-2, IL-6, IL-12, IL-17, IL-23, IL-33, TNF-, IFN-

• #48 Learn About Behcet’s Disease Symptoms & Manifestations, Causes, and Management | Behcet’s Connection – Celgene Corporation

  https://www.behcetsconnection.com/about-behcets

  No single underlying cause of Behet’s Disease has been identified. It is thought that genetic predisposition and environmental triggers may work together to activate an autoimmune cascade that leads to Behet’s Disease. The exact mechanism of the inflammatory changes in Behet’s Disease remains unknown. […] Environmental Triggers […] Viral or bacterial agents such as Streptococcus sanguinis, herpes simplex virus, Epstein-Barr virus, and cytomegalovirus […] Genetic Predisposition […] Family history […] HLA-B51 […] IL-10 […] IL23R/IL12RB2 […] Immune Response Activation […] Increased Production Of Pro-inflammatory Cytokines […] IL-2, IL-6, IL-12, IL-17, IL-23, IL-33, TNF-, IFN-

• #49 Behçet’s disease in children, an overview | Pediatric Rheumatology | Full Text

  https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-016-0070-z

  The aetiology of BD is still unknown; however, it appears to be a complex disease relying on an interaction between the genetic background and the environment. […] The underlying mechanisms causing the disease are still unclear and could combine both autoinflammation and autoimmunity. […] The recent discovery of A20 protein haploinsufficiency as a cause of autosomal dominant Behet-like disease strongly supports the idea that the full spectrum of disorders meeting criteria for BD cannot be related to a single mechanism. […] Pathogens such as herpes simplex virus, streptococci, staphylococci, or Escherichia species, and heat-shock proteins may contribute to attacks of BD in susceptible persons via stimulation of the innate immune system (inflammasome activation) through TLR2 and TLR4. […] Improvement of dental and periodontal hygiene has been associated with a decrease of oral ulcerations, and could be one factor explaining the decrease of the incidence of BD in countries like Japan and Turkey.

• #50 Pathogenesis of Behçet syndrome – UpToDate

  https://www.uptodate.com/contents/pathogenesis-of-behcet-syndrome

  The etiology and pathogenesis of Behçet syndrome are discussed in this review. […] The underlying cause of Behçet syndrome is unknown. As with other autoimmune diseases, the disorder may represent aberrant immune activity triggered by exposure to an agent, perhaps infectious, in patients with a genetic predisposition to develop the disease. […] Major disease mechanisms in Behçet syndrome include the following: Genetic influences, including association with certain human leukocyte antigens (HLA) as well as some non-HLA genes.

• #51

  https://journals.lww.com/neur/fulltext/2018/66060/focus_on_neuro_beh_et_s_disease__a_review.17.aspx

  Behet’s disease (BD) is a multisystemic vasculitis disorder of almost unknown etiology, which involves small and large vessels and affects both veins and arteries. […] The exact pathogenesis of BD is still unclear, but the main histopathological feature is a widespread vasculitis of arteries or venules of any size which can involve nearly every system and organ, such as the gastrointestinal tract, large vessels (veins and/or arteries), heart, and rarely, kidney. […] A genetic contribution has been found; human leucocytic antigen (HLA)-B5 allele, and, more specifically, HLA-B51, was found to be the most strong genetic factor related to the onset of BD (even if it accounts for less than 20% of cases and other genetic factors remain to be discovered). […] As far as etiopathogenesis is concerned, the etiologic factors of BD remain largely unknown.

• #52 Behcet Disease – EyeWiki

  https://eyewiki.org/Behcet_Disease

  There are many postulated genetic and environmental factors that are presumed to play a role. Abnormal immune activity may be generated by exposure to infectious (viral and bacterial) or non-infectious (environmental sources, such as heavy metals or chemicals) antigens in patients with a genetic predisposition.

• #53 Behcet’s Disease – Rheumatology Advisor

  https://www.rheumatologyadvisor.com/ddi/behcets-disease/

  Behets disease causes inflammation in blood vessels throughout the body, with veins more commonly affected than arteries. […] The management of Behets disease aims to control symptoms and prevent organ damage by decreasing inflammation and/or suppressing the immune system. […] Behets disease is a chronic inflammatory disorder affecting blood vessels in multiple body systems, with veins more commonly affected than arteries.

• #54 Behcets Disease General Information Research Page

  https://behcets.info/

  Behet’s disease (BD) is a chronic, relapsing, and debilitating systemic vasculitis of unknown aetiology with the clinical features of mucocutaneous lesions, ocular, vascular, articular, neurologic, gastrointestinal, urogenital, and pulmonary involvement. […] The most probable hypothesis is that of an inflammatory reaction set off by infectious agents such as herpes simplex virus 1 or Streptococcus spp. or by an autoantigen such as heat shock proteins in genetically predisposed individuals. […] The diagnosis is based on clinical criteria, as there is no pathognomonic test. […] Although several immunological abnormalities have been demonstrated, the exact mechanism of the inflammatory changes occurring remains to be elucidated. […] The disease is particularly prevalent in Silk Route populations but has global distribution.

• #55 Behçet disease: Background, Treatment and More

  https://dermnetnz.org/topics/behcet-disease

  Behet disease is of unknown cause, but it is thought to have an autoimmune basis. It is associated with several genetic factors, particularly HLA-B51. Several infections have also been implicated as potential triggers including Streptococcus sanguis, herpes simplex virus, hepatitis viruses, and parvovirus B19. […] Behet disease typically runs a relapsing-remitting course and the prognosis varies depending on the organ systems affected.

• #56 Autoimmune Skin Conditions: Types, Symptoms, Causes

  https://www.verywellhealth.com/behcets-disease-4802361

  Behcet’s disease is a rare disorder that causes blood vessel inflammation throughout the body. The condition causes mouth sores, eye inflammation, skin rashes, and genital lesions. […] Behcets disease affects 0.33 to 5.2 people per 100,000 in the United States. Anyone can develop Behcets disease at any age, but symptoms often start between ages 20 and 30 and are more common in men and in the Middle East and certain Asian countries. […] Behcets severity varies from person to person. Symptoms can come and go and people with the condition may experience periods of remission (where the disease stops or slows down) and periods of a flare-up (high disease activity). While the condition cannot be cured, various treatments can help to manage it.

• #57

  https://link.springer.com/article/10.1007/s10238-004-0033-4

  Behcets disease (BD) is a systemic disorder of recurrent acute inflammation, characterized by major symptoms of oral aphthous ulcers, uveitis, skin lesions and genital ulcers. […] Although etiology of the disease is still unknown, high prevalence of HLA-B51, increased expression of heat shock protein 60 and Th1 dominant immune responses in the patients are considered important in its pathogenesis. […] Non-infectious neutrophil activation and infection with Streptococcus sanguis and herpes simplex virus would also be associated. […] Further studies for elucidation of the etiology, improvement of the diagnostic criteria and development of new therapy are needed to conquer the disease.

• #58 Behçet’s Disease: Epidemiology, Etiopathogenesis, Diagnosis and Treatment | Frontiers Research Topic

  https://www.frontiersin.org/research-topics/13770/behcets-disease-epidemiology-etiopathogenesis-diagnosis-and-treatmentundefined

  Behet’s disease (BD) is a chronic, relapsing inflammatory, multisystem disease of unknown etiology with the clinical features of mucocutaneous lesions and ocular, vascular, articular, neurologic, gastrointestinal, urogenital, pulmonary, and cardiac involvement. […] Advances in understanding the etiopathogenesis and genetics of BD help us to facilitate the development of agents targeting specific inflammatory responses in the disease. Several recent GWASs have confirmed the association of BD with HLA-B*51 and reported new susceptibility genes both on the remaining part of HLA Class I region and on non-HLA genes.

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