Materiały źródłowe

• #1 Giant cell arteritis // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/giant-cell-arteritis

  Giant cell arteritis causes inflammation of certain arteries, especially those near the temples. […] What causes these arteries to become inflamed isn’t known, but it’s thought to involve abnormal attacks on artery walls by the immune system. Certain genes and environmental factors might increase your susceptibility to the condition.

• #1 Giant Cell Arteritis (Temporal Arteritis): Signs & Treatment

  https://my.clevelandclinic.org/health/diseases/temporal-arteritis-giant-cell-arteritis

  Giant cell arteritis (GCA), previously known as temporal arteritis, is a form of vasculitis (inflammation of your blood vessels). It affects the large blood vessels in your body, particularly the arteries in your head, neck and arms. […] Researchers dont know the exact cause of giant cell arteritis, but many believe its an autoimmune or autoinflammatory disease. This means your bodys immune system attacks your healthy blood vessels. Because the condition mainly affects people as they age, some believe it could be linked to the aging process. They also believe genetics and environmental factors that stress your immune system may play a role.

• #1 Temporal arteritis: Symptoms, diagnosis, and causes

  https://www.medicalnewstoday.com/articles/312614

  Temporal arteritis, now known as giant cell arteritis, is a form of vasculitis, or blood vessel inflammation. […] It is an auto-immune condition that happens because of a faulty immune system reaction, when the immune system attacks healthy cells by mistake. […] The exact cause of GCA is unknown, but it is thought to be an auto-immune condition, in which the bodys immune system attacks healthy cells in the arteries. This leads to inflammation. […] When the blood vessel lining becomes inflamed, giant cell lesions can form. […] These giant cells form when numerous immune cells fuse together. These immune cells are a type of white blood cell.

• #1 Giant Cell Arteritis (Temporal Arteritis) – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK459376/

  Although the exact etiology is unknown, researchers have associated the disease with several genetic and environmental factors. Large-scale genetic analyses reveal a strong association between GCA and specific polymorphisms in the major histocompatibility complex region, such as human leukocyte antigen (HLA)-DRB1*04:04, HLA-DQA1*03:01, and HLA-DQB1*03:02. […] GCA is a granulomatous vasculitis believed to occur as an immune-mediated disease leading to the activation of vascular dendritic cells. Activated dendritic cells produce chemokines, such as granulocyte-macrophage colony-stimulating factor, that attract and retain dendritic cells, lymphocytes, and macrophages. […] Although numerous triggers, including various pathogens, have been suggested, researchers have not definitively established a causal link between these triggers and GCA.

• #1 Giant Cell Arteritis – Rheumatology Advisor

  https://www.rheumatologyadvisor.com/ddi/giant-cell-arteritis/

  The main clinical risk factor for GCA is age-related predisposition. This could be linked to concurrent aging of the immune system and the blood vessel wall, where dendritic cells are generally found. Other risk factors for giant cell arteritis are smoking history and increased diastolic blood pressure. […] Some unknown triggers cause abnormal maturation of vascular dendritic cells in the adventitia of large vessel walls. Cluster differentiation (CD) 4+ naive T lymphocytes are recruited and activated by these activated dendritic cells. […] According to genome-wide association studies, HLA genes, such as HLA Br1*04 in the White population, are linked to GCA and increased risk of complications, including loss of vision and increased resistance to glucocorticoids. PTPN22, LRRC32, IL17A, and IL33 are examples of non-HLA genes linked to giant cell arteritis. The majority of the genes discovered are related to endothelial function, innate immunity, and cytokines and their receptors. […] GCA is believed to be an autoimmune disorder in which the body’s immune system attacks its own healthy tissues, leading to inflammation of the arteries. Age-related predisposition is the major risk factor for GCA.

• #1 Giant Cell Arteritis – EyeWiki

  https://eyewiki.org/Giant_Cell_Arteritis

  Giant cell arteritis (GCA) is the most common primary vasculitis in adults. […] The underlying etiology of GCA is complex and has been widely researched, yet is still not well understood. This includes genetic and possibly infectious factors, which go on to trigger an immune response. […] A genetic predisposition for GCA has been suspected, due to increased reports of GCA among first degree relatives and rare familial forms of GCA. […] Certain genes within the human leukocyte antigen (HLA) class I and class II regions, specifically, HLA DRB1*04, DRW6, and DR3 have been associated with increased susceptibility to GCA as well. […] Other linked genes are those related to cytokine and chemokine expression, which can alter the clinical presentation of GCA in different patients. […] In addition to genetic factors, an infectious or environmental factor has been suspected due to successive studies of a stable population in Minnesota, where a cyclic fluctuation in the incidence of GCA was noted.

• #1 Giant Cell Arteritis (Temporal Arteritis): Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/332483-overview

  A possible association between Toll-like receptor 4 gene polymorphism and susceptibility to biopsy-proven GCA has been found. […] Epidemiological observations, reports, and studies using DNA detection techniques have implicated Chlamydia pneumoniae, Mycoplasma pneumoniae, parvovirus B19, and varicella zoster as the impetus for the destructive inflammation. […] Nevertheless, it is generally accepted that these infectious agents are only „innocent bystanders.” […] The immune system (both cellular and humoral) has been implicated in the pathogenesis of GCA. The granulomatous histopathology of GCA has suggested the presence of an antigen-driven disease with local T-cell and macrophage activation in or near elastic tissue in the arterial walls with an important role of the proinflammatory cytokines.

• #1 Giant cell arteritis – Wikipedia

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

  The cause is unknown. […] The underlying mechanism involves inflammation of the small blood vessels that supply the walls of larger arteries. […] The varicella-zoster virus (VZV) antigen was found in 74% of temporal artery biopsies that were GCA-positive, suggesting that the VZV infection may trigger the inflammatory cascade. […] The pathological mechanism is the result of an inflammatory cascade that is triggered by an as of yet undetermined cause resulting in dendritic cells in the vessel wall recruiting T cells and macrophages to form granulomatous infiltrates.

• #1 Giant Cell Arteritis: Signs, Symptoms, Causes & Treatments

  https://www.acko.com/health-insurance/giant-cell-arteritis/

  Giant Cell Arteritis is a type of vasculitis that causes inflammation of blood vessels, specifically of the arteries in the head, neck, and upper limbs. It is also called temporal arteritis. This inflammation can cause damage to the artery walls leading to narrowing, constriction, and hardening of the artery. If left untreated, the condition can lead to permanent and severe vision loss. […] The exact cause of GCA is unknown, but it is believed that genetic and environmental factors both play a role. […] Genetic factors are thought to be an important contributor to GCA. Several genetic traits have been linked to GCA, including certain HLA alleles, or genetic markers, and a gene known as CDKN2A, which is associated with an increased risk of this condition. […] Environmental factors, such as smoking, air pollution, and exposure to certain viruses or bacteria, have been linked to an increased risk of GCA. Also, people with autoimmune conditions, such as rheumatoid arthritis, may be more likely to develop GCA.

• #1 Eyes – giant cell arteritis | Better Health Channel

  https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/eyes-giant-cell-arteritis

  Giant cell arteritis is inflammation of the arteries that can cause sudden blindness in one or both eyes. […] People over the age of 50 years are at risk of developing the disease, for reasons unknown. […] The causes of giant cell arteritis are unknown. Some researchers believe that it may be a type of autoimmune disorder. […] Risk factors include: Gender twice as many women as men are affected. Age people over the age of 50 years are more susceptible. The average age at diagnosis is 70 years. Race giant cell arteritis is more common among Caucasians. Region prevalence is higher in northern European nations.

• #1 Temporal Arteritis Pathology: Definition, Epidemiology, Etiology

  https://emedicine.medscape.com/article/1612591-overview

  The etiology of temporal arteritis is multifactorial and is determined by both environmental and genetic factors. Data indicate that the disease is probably initiated by exposure to an exogenous antigen. Numerous viruses and bacteria have been proposed as potential precipitants, including parvovirus, parainfluenza virus, varicella-zoster virus, […] Chlamydia pneumoniae, and Mycoplasma pneumoniae. […] Both cellular and humoral immune systems contribute to the immunopathology of giant cell arteritis (GCA). Key events include the activation of vascular dendritic cells located in the adventitia-media junction, which trigger immune responses by presenting antigens and recruiting CD4+T helper cells to the vessel wall. Activated CD4+ T helper cells develop into two distinct T cell lineages Th1 and Th17 that produce various pro-inflammatory cytokines.

• #1 Giant Cell Arteritis – EyeWiki

  https://eyewiki.org/Giant_Cell_Arteritis

  A range of infectious stimuli have been implicated, including Chlamydia pneumoniae, varicella virus, and parvovirus B19. […] After the initial trigger, regardless of etiology, a dual immune response begins. […] GCA preferentially affects three-layered vessels. […] The primary site of injury is in the adventitial layer. […] Activated T-cells in the vasa vasorum of medium and large vessels up-regulate and activate macrophages, which then form a granulomatous immune reaction. […] This results in the destruction and remodeling of the arterial wall and progressive occlusion of the lumen responsible for the ischemic symptoms of GCA. […] Metalloproteinases and reactive oxygen intermediates expressed by macrophages ultimately cause destruction within the blood vessel wall. […] There has been no evidence demonstrating the role of B cells in the development of GCA.

• #1 Giant Cell Arteritis: Advances in Understanding Pathogenesis and Implications for Clinical Practice

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10855045/

  Regarding epigenetic modifications that have been shown to be involved in the onset of GCA, hypomethylation of loci of genes encoding for proteins involved in T-cell receptor (TCR) activation, especially after interaction with the co-stimulatory molecule CD28 and of genes involved in the calcineurin-mediated intracellular pathway, which is critical for the induction of the nuclear transcription factor of activated T cells (NFAT), was observed in the arterial wall of GCA patients. […] Another epigenetic factor reported to be possibly involved in the genesis of GCA is the microbiota. […] The first pathogen thought to play a crucial role in the pathogenesis of GCA was the varicella-zoster virus (VZV).

• #1 Giant cell arteritis: reviewing the advancing diagnostics and management | Eye

  https://www.nature.com/articles/s41433-023-02433-y

  Giant Cell Arteritis (GCA) is well known to be a critical ischaemic disease that requires immediate medical recognition to initiate treatment and where one in five people still suffer visual loss. […] The immunopathophysiology has continued to be characterised, and the influencing of ageing in the development of GCA is beginning to be understood. […] A number of risk factors for the development of GCA have been identified such as history of vascular disease, smoking, low body mass index and early menopause, however ageing has been found to be the strongest of all risk factors. […] GCA has been reliably associated with major histocompatibility complex molecules (i.e. HLA-DR3, HLA-DR4, HLA-DR5 and HLA-DRB1) particularly with carriage of HLA-DRB1*04 alleles. […] Ageing has been known to make blood vessels vulnerable to damage and inflammation, with coining of the term inflammaging and atherosclerosis being described as a prototypical form of vascular ageing.

• #1 Giant cell arteritis: reviewing the advancing diagnostics and management | Eye

  https://www.nature.com/articles/s41433-023-02433-y

  The link between GCA and atherosclerosis remains ambiguous. […] The underlying protective mechanism of atherosclerosis and GCA currently remains unclear, however hyperglycaemia has been speculated to impair T-cell function, suppressing the inflammatory response in GCA. […] GCA predominantly affects people 50 years of age, with rising prevalence in the context of an aging population and peak in the 7th decade. […] Population studies have observed that ageing is associated with chronically higher circulating levels of pro-inflammatory cytokines and inflammatory markers, namely, IL-6, IL-18, IL-1ra, C-reactive protein (CRP), and fibrinogen. […] IL-6 has been associated with clonal hematopoiesis of indeterminate potential (CHIP), a pre-malignant state characterised by somatic mutations in hematologic precursor cells is another potential pathogenetic mechanism potentially implicated in development of GCA.

• #1 Giant cell arteritis: reviewing the advancing diagnostics and management | Eye

  https://www.nature.com/articles/s41433-023-02433-y

  Preliminary works exploring a potential correlation between CHIP and the development of GCA seem to corroborate this association. […] Another theory is the potential role of somatic variants (SV) in GCA, as the number of SVs increases with ageing. […] SVs can render immune system cells resistant to apoptosis or change their functional profile (i.e. leading to aberrant cytokine secretion), causing high-inflammatory, non-proliferative (i.e. non-neoplastic) immune disorders.

• #1 Giant Cell Arteritis — RheumInfo

  https://rheuminfo.com/en/diseases/giant-cell-arteritis/

  Scientists dont fully understand what causes giant cell arteritis, but researchers around the world are actively studying this condition to learn more about it. […] Its known that parts of the immune system are activated or (turned on) in people with giant cell arteritis and other types of vasculitis (diseases involving inflamed blood vessels). […] One possibility is that the immune system is triggered by an infection and starts to attack the arteries. If this is the case, the source of such an infection has yet to be identified. […] Giant Cell Arteritis is sometimes associated with another rheumatic disease called Polymyalgia Rheumatica (PMR). Up to 70% of the people who have giant cell arteritis also have Polymyalgia Rheumatica.

• #1 Giant Cell Arteritis Causes, Symptoms, and Treatments

  https://www.upmc.com/services/heart-vascular/conditions/giant-cell-arteritis

  Giant cell arteritis is a rare type of vasculitis, or inflammation in the blood vessels. […] While the cause of giant cell arteritis is unknown, doctors believe the following play a role in causing it: Inflammation, Possible infection, Age, Gender. […] Some risk factors of giant cell arteritis can include: Age – People over 50 are more likely to have this condition. Gender – Women are more likely than men to get giant cell arteritis. […] If left untreated, giant cell arteritis can lead to serious and even life-threatening complications like permanent blindness and stroke. […] Treatment for giant cell arteritis usually includes steroid medication to reduce inflammation. […] Also, giant cell arteritis can recur even after treatment.

• #2 Giant Cell Arteritis (Temporal Arteritis): Signs & Treatment

  https://my.clevelandclinic.org/health/diseases/temporal-arteritis-giant-cell-arteritis

  Giant cell arteritis (GCA), previously known as temporal arteritis, is a form of vasculitis (inflammation of your blood vessels). It affects the large blood vessels in your body, particularly the arteries in your head, neck and arms. […] Researchers dont know the exact cause of giant cell arteritis, but many believe its an autoimmune or autoinflammatory disease. This means your bodys immune system attacks your healthy blood vessels. Because the condition mainly affects people as they age, some believe it could be linked to the aging process. They also believe genetics and environmental factors that stress your immune system may play a role.

• #2 Giant cell arteritis (GCA) | Causes, symptoms, treatments

  https://versusarthritis.org/about-arthritis/conditions/giant-cell-arteritis-gca/

  GCA is an autoimmune condition. This is when your immune system, which is your body’s defence system that fights infections, gets confused and attacks healthy tissues in the body. […] In people who have GCA, the immune system mistakenly attacks healthy arteries. […] It may be that the genes you inherited from your parents made you more likely to develop GCA. However, just because someone has GCA, it doesn’t mean their children will definitely get it.

• #2 Giant cell arteritis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/giant-cell-arteritis?lang=us

  Giant cell arteritis (GCA) is a common granulomatous vasculitis affecting medium to large-sized arteries. […] It is histologically similar to other large vessel vasculitides (such as Takayasu arteritis) showing granulomatous inflammation of arteries with infiltration predominantly by histiocytes, lymphocytes, and multinucleated giant cells. […] The characteristic multinucleated giant cells are only found in ~50% of cases.

• #2 Giant Cell Arteritis – EyeWiki

  https://eyewiki.org/Giant_Cell_Arteritis

  Giant cell arteritis (GCA) is the most common primary vasculitis in adults. […] The underlying etiology of GCA is complex and has been widely researched, yet is still not well understood. This includes genetic and possibly infectious factors, which go on to trigger an immune response. […] A genetic predisposition for GCA has been suspected, due to increased reports of GCA among first degree relatives and rare familial forms of GCA. […] Certain genes within the human leukocyte antigen (HLA) class I and class II regions, specifically, HLA DRB1*04, DRW6, and DR3 have been associated with increased susceptibility to GCA as well. […] Other linked genes are those related to cytokine and chemokine expression, which can alter the clinical presentation of GCA in different patients. […] In addition to genetic factors, an infectious or environmental factor has been suspected due to successive studies of a stable population in Minnesota, where a cyclic fluctuation in the incidence of GCA was noted.

• #2 Temporal Arteritis Pathology: Definition, Epidemiology, Etiology

  https://emedicine.medscape.com/article/1612591-overview

  Individuals homozygous for HLA-DR4 and the FCGR2A-131RR allele have a sixfold greater risk for temporal arteritis. HLA-DR1, HLA-DR3, and HLA-DR5 may also be associated with a higher incidence of the disease. Genome-wide association studies have identified HLA-DRB1*04, as the most common gene among Caucasians associated with a higher risk for temporal arteritis, resistance to glucocorticoid therapy, and greater incidence of complications such as visual loss. […] The precise role of genetic susceptibility in the pathogenesis of temporal arteritis remains unclear and requires further investigation.

• #2 Giant Cell Arteritis: Advances in Understanding Pathogenesis and Implications for Clinical Practice

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10855045/

  Giant cell arteritis (GCA) is a noninfectious granulomatous vasculitis of unknown etiology affecting individuals older than 50 years. […] Although the clinical and epidemiological features of GCA have been partly elucidated, the causes of this vasculitis remain largely unknown. […] Several epigenetic (micro)environmental factors are often critical for the onset of this vasculitis. […] A key role in the pathogenesis of GCA is played by cells of both the innate and adaptive immune systems, which contribute to the formation of granulomas that may include giant cells, a hallmark of the disease, and arterial tertiary follicular organs. […] Early studies on the genetic predisposition to develop GCA focused on the association with the presence of particular HLA alleles. […] New associations between genetic mutations and GCA have also been described.

• #2 Giant Cell Arteritis: Signs, Symptoms, Causes & Treatments

  https://www.acko.com/health-insurance/giant-cell-arteritis/

  Giant Cell Arteritis is a type of vasculitis that causes inflammation of blood vessels, specifically of the arteries in the head, neck, and upper limbs. It is also called temporal arteritis. This inflammation can cause damage to the artery walls leading to narrowing, constriction, and hardening of the artery. If left untreated, the condition can lead to permanent and severe vision loss. […] The exact cause of GCA is unknown, but it is believed that genetic and environmental factors both play a role. […] Genetic factors are thought to be an important contributor to GCA. Several genetic traits have been linked to GCA, including certain HLA alleles, or genetic markers, and a gene known as CDKN2A, which is associated with an increased risk of this condition. […] Environmental factors, such as smoking, air pollution, and exposure to certain viruses or bacteria, have been linked to an increased risk of GCA. Also, people with autoimmune conditions, such as rheumatoid arthritis, may be more likely to develop GCA.

• #2 Giant Cell Arteritis (Temporal Arteritis): Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/332483-overview

  Giant cell arteritis (GCA), or temporal arteritis, is a systemic inflammatory vasculitis of unknown etiology that occurs in older persons and can result in a wide variety of systemic, neurologic, and ophthalmologic complications. […] Age and female sex are established risk factors for GCA, a genetic component seems likely, and infection may have a role (see Etiology). […] The exact etiology of giant cell arteritis (GCA) remains unknown. Despite increased understanding of the inflammatory cascade responsible for the disease process, the initial event that triggers the cascade remains uncertain. Genetic, environmental, and autoimmune factors have been identified. […] Reports of familial aggregation, association with the HLA-DR4 haplotype, and an apparent higher frequency of these conditions in northern Europe and in persons in the United States with similar ethnic backgrounds suggest a genetic or hereditary predisposition.

• #2 Giant Cell Arteritis – EyeWiki

  https://eyewiki.org/Giant_Cell_Arteritis

  A range of infectious stimuli have been implicated, including Chlamydia pneumoniae, varicella virus, and parvovirus B19. […] After the initial trigger, regardless of etiology, a dual immune response begins. […] GCA preferentially affects three-layered vessels. […] The primary site of injury is in the adventitial layer. […] Activated T-cells in the vasa vasorum of medium and large vessels up-regulate and activate macrophages, which then form a granulomatous immune reaction. […] This results in the destruction and remodeling of the arterial wall and progressive occlusion of the lumen responsible for the ischemic symptoms of GCA. […] Metalloproteinases and reactive oxygen intermediates expressed by macrophages ultimately cause destruction within the blood vessel wall. […] There has been no evidence demonstrating the role of B cells in the development of GCA.

• #2 Temporal Arteritis: Symptoms, Treatments, and Causes

  https://www.healthline.com/health/temporal-arteritis

  Temporal arteritis has no definitive cause, but researchers speculate that a viral or bacterial infection may be the trigger in many cases. […] Some of the suspected viruses and bacteria that may be triggers include: varicella-zoster virus (VZV), which causes chickenpox and shingles, herpes simplex virus, EpsteinBarr virus, parvovirus B19, chlamydia pneumoniae, mycoplasma pneumoniae. […] However, there is no evidence to definitively prove or disprove this. Temporal arteritis, once it occurs, appears to be autoimmune in nature. This means that the body’s own immune cells attack healthy tissue in the artery walls.

• #2 Giant cell arteritis | MedLink Neurology

  https://www.medlink.com/articles/giant-cell-arteritis

  Giant cell arteritis, also called temporal arteritis, is the most common form of systemic vasculitis in the elderly. Its etiology is unknown. Advanced age and Caucasian race are risk factors. […] The pathogenesis of giant cell arteritis is believed to be autoimmune. The cellular infiltrate is dominated by mononuclear cells and multinucleated giant cells. Monocyte to macrophage differentiation plays an important role. Two pathogenetic mechanisms are postulated. One mechanism is mediated by Th17 T-cells that produce interleukin 17, which leads to systemic inflammation manifest as steroid-responsive fever, malaise, anorexia, and headache. The other mechanism is mediated by Th1 T-cells that produce interferon-gamma and steroid-resistant vascular wall inflammation. […] Varicella zoster virus antigen and DNA have been found in localized patches of arteries that were called skip areas in pathologically negative temporal artery specimens examined in patients clinically diagnosed with giant cell arteritis.

• #2 Giant Cell Arteritis (Temporal Arteritis): Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/332483-overview

  A possible association between Toll-like receptor 4 gene polymorphism and susceptibility to biopsy-proven GCA has been found. […] Epidemiological observations, reports, and studies using DNA detection techniques have implicated Chlamydia pneumoniae, Mycoplasma pneumoniae, parvovirus B19, and varicella zoster as the impetus for the destructive inflammation. […] Nevertheless, it is generally accepted that these infectious agents are only „innocent bystanders.” […] The immune system (both cellular and humoral) has been implicated in the pathogenesis of GCA. The granulomatous histopathology of GCA has suggested the presence of an antigen-driven disease with local T-cell and macrophage activation in or near elastic tissue in the arterial walls with an important role of the proinflammatory cytokines.

• #2 Giant Cell Arteritis – Rheumatology Advisor

  https://www.rheumatologyadvisor.com/ddi/giant-cell-arteritis/

  The main clinical risk factor for GCA is age-related predisposition. This could be linked to concurrent aging of the immune system and the blood vessel wall, where dendritic cells are generally found. Other risk factors for giant cell arteritis are smoking history and increased diastolic blood pressure. […] Some unknown triggers cause abnormal maturation of vascular dendritic cells in the adventitia of large vessel walls. Cluster differentiation (CD) 4+ naive T lymphocytes are recruited and activated by these activated dendritic cells. […] According to genome-wide association studies, HLA genes, such as HLA Br1*04 in the White population, are linked to GCA and increased risk of complications, including loss of vision and increased resistance to glucocorticoids. PTPN22, LRRC32, IL17A, and IL33 are examples of non-HLA genes linked to giant cell arteritis. The majority of the genes discovered are related to endothelial function, innate immunity, and cytokines and their receptors. […] GCA is believed to be an autoimmune disorder in which the body’s immune system attacks its own healthy tissues, leading to inflammation of the arteries. Age-related predisposition is the major risk factor for GCA.

• #2 Clinical manifestations of giant cell arteritis – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-of-giant-cell-arteritis

  Giant cell arteritis (GCA, also known as Horton disease, cranial arteritis, and temporal arteritis) is categorized as a vasculitis of large- and medium-sized vessels because it can involve the aorta and great vessels. […] The greatest risk factor for developing GCA is aging. The disease almost never occurs before age 50 years, and its incidence rises steadily thereafter, peaking between the ages of 70 to 79, with over 80 percent of patients older than 70 years of age. […] In addition to age, ethnicity is a major risk factor for GCA. The highest incidence figures are found in Scandinavian countries and among Americans of Scandinavian descent. […] Autopsy studies suggest that GCA is more frequent than reported in studies of clinically diagnosed cases. […] Most studies have found that life expectancy is not, or is only marginally, reduced in GCA, with the exception of the subset of patients who develop aortic aneurysm or aortic dissection or rupture.

• #2 Giant Cell Arteritis Causes, Symptoms, and Treatments

  https://www.upmc.com/services/heart-vascular/conditions/giant-cell-arteritis

  Giant cell arteritis is a rare type of vasculitis, or inflammation in the blood vessels. […] While the cause of giant cell arteritis is unknown, doctors believe the following play a role in causing it: Inflammation, Possible infection, Age, Gender. […] Some risk factors of giant cell arteritis can include: Age – People over 50 are more likely to have this condition. Gender – Women are more likely than men to get giant cell arteritis. […] If left untreated, giant cell arteritis can lead to serious and even life-threatening complications like permanent blindness and stroke. […] Treatment for giant cell arteritis usually includes steroid medication to reduce inflammation. […] Also, giant cell arteritis can recur even after treatment.

• #2 Temporal Arteritis Pathology: Definition, Epidemiology, Etiology

  https://emedicine.medscape.com/article/1612591-overview

  The access of monocytes and T cells to the vascular wall is controlled by matrix metalloproteinase (MMP)-9, a type IV collagenase that is produced in the vasculitic lesions of giant cell arteritis. MMP-9 production allows T cells to pass through collagen IVcontaining basement membrane. Adventitia-based macrophages produce interleukin-6 (IL-6), which further augments the inflammatory cascade. […] The exuberant release of cytokines associated with this process may be responsible for the constitutional symptoms frequently encountered with the disease. Proinflammatory cytokine IL-6, responsible for the exaggerated acute phase of the immune response, is implicated in high levels of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). […] On the basis of its familial, ethnic, and geographic distribution, temporal arteritis appears to have a genetic predisposition. Studies have shown familial clustering and monozygotic twin concordance. Most genetic factors center on the human leukocyte antigen (HLA) genes. It is likely that various HLA alleles predispose to temporal arteritis and mediate its severity.

• #2 Giant cell arteritis: reviewing the advancing diagnostics and management | Eye

  https://www.nature.com/articles/s41433-023-02433-y

  The link between GCA and atherosclerosis remains ambiguous. […] The underlying protective mechanism of atherosclerosis and GCA currently remains unclear, however hyperglycaemia has been speculated to impair T-cell function, suppressing the inflammatory response in GCA. […] GCA predominantly affects people 50 years of age, with rising prevalence in the context of an aging population and peak in the 7th decade. […] Population studies have observed that ageing is associated with chronically higher circulating levels of pro-inflammatory cytokines and inflammatory markers, namely, IL-6, IL-18, IL-1ra, C-reactive protein (CRP), and fibrinogen. […] IL-6 has been associated with clonal hematopoiesis of indeterminate potential (CHIP), a pre-malignant state characterised by somatic mutations in hematologic precursor cells is another potential pathogenetic mechanism potentially implicated in development of GCA.

• #2 Giant cell arteritis: reviewing the advancing diagnostics and management | Eye

  https://www.nature.com/articles/s41433-023-02433-y

  Preliminary works exploring a potential correlation between CHIP and the development of GCA seem to corroborate this association. […] Another theory is the potential role of somatic variants (SV) in GCA, as the number of SVs increases with ageing. […] SVs can render immune system cells resistant to apoptosis or change their functional profile (i.e. leading to aberrant cytokine secretion), causing high-inflammatory, non-proliferative (i.e. non-neoplastic) immune disorders.

• #2 Giant Cell Arteritis | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions/g/giant-cell-arteritis.html

  Giant cell arteritis (GCA) is the inflammation of an artery a blood vessel that carries oxygen-rich blood to the body and it can occur anywhere within the body. […] The cause of GCA is unknown. Because the condition almost always occurs in people over the age of 50 (the average age of patients with GCA is about 70), it may be related to the aging process. Genetic factors may also be involved since the condition is more commonly found in patients of Northern European ancestry. GCA occurs twice as often in women as in men. […] The condition is also associated with polymyalgia rheumatica. About 15 percent of patients who have the condition are also diagnosed with GCA.

• #2 Understanding Temporal Arteritis: Symptoms, Causes & Treatments

  https://conloneyeinstitute.com/understanding-temporal-arteritis-symptoms-causes-treatments/

  Proinflammatory cytokines play a significant role in GCA pathogenesis, and the condition can also increase the risk of cardiovascular disease. […] GCA is associated with age, gender, genetics, smoking, and dysregulation of the immune system, all of which can increase one’s risk of developing this condition.

• #2 Temporal Arteritis (Giant Cell Arteritis)

  https://patient.info/eye-care/visual-problems/temporal-arteritis-giant-cell-arteritis

  Temporal arteritis, also known as giant cell arteritis (GCA), is a condition which causes inflammation on the inside of some blood vessels (arteries). It is called 'giant cell’ because abnormal large cells develop in the wall of the inflamed arteries. The cause is not known. […] GCA is uncommon and mainly affects people over the age of 60 years. It rarely affects people aged under 50 years. Women are more commonly affected than men. […] The typical symptoms of PMR are pain, tenderness and stiffness of muscles around the shoulders and upper arms, and sometimes around the hips and neck. PMR is due to inflammation in the affected muscles, but the cause is unknown. […] Possible complications include the following: […] If an affected blood vessel (artery) becomes very swollen (inflamed), the blood supply going down that artery can become blocked. […] Other serious complications sometimes develop if the inflammation occurs in other arteries. For example, serious complications can include a heart attack, an aortic aneurysm, a stroke, damage to nerves, or deafness (caused by a blocked artery in the brain).

• #3 Giant Cell Arteritis (Temporal Arteritis): Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/332483-overview

  Giant cell arteritis (GCA), or temporal arteritis, is a systemic inflammatory vasculitis of unknown etiology that occurs in older persons and can result in a wide variety of systemic, neurologic, and ophthalmologic complications. […] Age and female sex are established risk factors for GCA, a genetic component seems likely, and infection may have a role (see Etiology). […] The exact etiology of giant cell arteritis (GCA) remains unknown. Despite increased understanding of the inflammatory cascade responsible for the disease process, the initial event that triggers the cascade remains uncertain. Genetic, environmental, and autoimmune factors have been identified. […] Reports of familial aggregation, association with the HLA-DR4 haplotype, and an apparent higher frequency of these conditions in northern Europe and in persons in the United States with similar ethnic backgrounds suggest a genetic or hereditary predisposition.

• #3 Temporal Arteritis: Symptoms, Causes, Treatments

  https://www.webmd.com/heart-disease/arteritis-giant-cell

  Giant cell arteritis, also called temporal arteritis, is a disease that causes your arteries — blood vessels that carry oxygen from your heart to the rest of your body — to become inflamed. […] Its an autoimmune disease. That means your immune system mistakenly attacks your body’s healthy tissues. In giant cell arteritis, immune cells react against blood vessels and make them inflamed. […] Doctors don’t know what triggers this, but you’re more likely to get it if you’re: Over 50, Female, Of European descent — especially if your family is from a country in Scandinavia (Norway, Sweden, Denmark, or Finland).

• #3 Giant cell arteritis

  https://dermnetnz.org/topics/giant-cell-arteritis

  Giant cell arteritis is a large vessel vasculitis of unknown cause. […] Age, genetics, and infection may be implicated in its causation. […] The human leukocyte antigen (HLA) DR4 serotype has been correlated with giant cell arteritis and PMR.

• #3 Giant cell arteritis (temporal arteritis) Guide: Causes, Symptoms and Treatment Options

  https://www.drugs.com/health-guide/giant-cell-arteritis-temporal-arteritis.html

  Giant cell arteritis, also called temporal arteritis, is a disease in which the large arteries and their branches, including those that supply the eye, scalp, and face, become inflamed and narrowed. […] The cause of giant cell arteritis is unknown, although there has been speculation that in some people, the body’s immune system responds incorrectly to an infection, and begins to attack the lining of blood vessels as it would a foreign invader. […] There may be a genetic contribution as well, as suggested by occasional cases that affect more than one member of the same family. […] One possibility is that the immune system of certain individuals or family members may be genetically programmed to respond to an infection abnormally, a response that leads to giant cell arteritis.

• #3 Giant Cell Arteritis: Advances in Understanding Pathogenesis and Implications for Clinical Practice

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10855045/

  Regarding epigenetic modifications that have been shown to be involved in the onset of GCA, hypomethylation of loci of genes encoding for proteins involved in T-cell receptor (TCR) activation, especially after interaction with the co-stimulatory molecule CD28 and of genes involved in the calcineurin-mediated intracellular pathway, which is critical for the induction of the nuclear transcription factor of activated T cells (NFAT), was observed in the arterial wall of GCA patients. […] Another epigenetic factor reported to be possibly involved in the genesis of GCA is the microbiota. […] The first pathogen thought to play a crucial role in the pathogenesis of GCA was the varicella-zoster virus (VZV).

• #3 Understanding Temporal Arteritis: Symptoms, Causes & Treatments

  https://conloneyeinstitute.com/understanding-temporal-arteritis-symptoms-causes-treatments/

  Temporal arteritis, also called temporal arteritis, or giant cell arteritis (GCA), is a systemic inflammatory vasculitis affecting medium to large arteries, primarily in elderly individuals. […] Although age, ethnicity, and geography have been identified as potential risk factors, the primary cause of GCA is believed to be an inadequate response to endothelial injury, which may involve vascular endothelial growth factors. […] Genetic factors, such as low body mass index, early menopause, and relative adrenal hypofunction, have been linked to GCA. Additionally, smoking increases the risk of GCA by a factor of six in women. Research suggests that GCA is an antigen-driven disease characterized by its granulomatous histopathology. Local T-cell and macrophage activation can be seen in and around the elastic tissue of arterial walls.

• #3 Giant Cell Arteritis Causes, Symptoms, and Treatments

  https://www.upmc.com/services/heart-vascular/conditions/giant-cell-arteritis

  Giant cell arteritis is a rare type of vasculitis, or inflammation in the blood vessels. […] While the cause of giant cell arteritis is unknown, doctors believe the following play a role in causing it: Inflammation, Possible infection, Age, Gender. […] Some risk factors of giant cell arteritis can include: Age – People over 50 are more likely to have this condition. Gender – Women are more likely than men to get giant cell arteritis. […] If left untreated, giant cell arteritis can lead to serious and even life-threatening complications like permanent blindness and stroke. […] Treatment for giant cell arteritis usually includes steroid medication to reduce inflammation. […] Also, giant cell arteritis can recur even after treatment.

• #3 Temporal Arteritis Pathology: Definition, Epidemiology, Etiology

  https://emedicine.medscape.com/article/1612591-overview

  The etiology of temporal arteritis is multifactorial and is determined by both environmental and genetic factors. Data indicate that the disease is probably initiated by exposure to an exogenous antigen. Numerous viruses and bacteria have been proposed as potential precipitants, including parvovirus, parainfluenza virus, varicella-zoster virus, […] Chlamydia pneumoniae, and Mycoplasma pneumoniae. […] Both cellular and humoral immune systems contribute to the immunopathology of giant cell arteritis (GCA). Key events include the activation of vascular dendritic cells located in the adventitia-media junction, which trigger immune responses by presenting antigens and recruiting CD4+T helper cells to the vessel wall. Activated CD4+ T helper cells develop into two distinct T cell lineages Th1 and Th17 that produce various pro-inflammatory cytokines.

• #3 Giant cell arteritis – Knowledge @ AMBOSS

  https://www.amboss.com/us/knowledge/giant-cell-arteritis/

  Unknown; possible contributing factors include: genetic predisposition (e.g., human leukocyte antigen HLA-DR4) […] viral infections (e.g., parvovirus B19) […] Association with polymyalgia rheumatica (PMR): up to 50% of patients with giant cell arteritis also have PMR.

• #3 Giant Cell Arteritis : Johns Hopkins Vasculitis Center

  https://www.hopkinsvasculitis.org/types-vasculitis/giant-cell-arteritis/

  What Causes Giant Cell Arteritis? […] We do not know. We do know that aging has something to do with the disease. And we know that the bodys immune system attacks and inflames the arteries. But we do not know why the immune system attack occurs when and where it does. […] Giant cell arteritis is vasculitis of unknown cause that affects the elderly and is characterized by panarteritis of medium- to large-sized arteries, especially in the extracranial branches of the carotid artery. […] Because blindness from giant cell arteritis is almost irreversible, treatment with 40 to 60 mg of prednisone should be started as soon as the diagnosis is suspected.

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