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Arteritis

Arteritis is a medical condition characterized by inflammation of the arterial walls, which can lead to thickening, narrowing (stenosis), weakening, or aneurysm formation, thereby restricting blood flow and potentially damaging organs supplied by the affected arteries.^[1] It represents a subset of vasculitis, specifically involving medium- and large-sized arteries, and includes several distinct primary forms such as giant cell arteritis (GCA), Takayasu arteritis (TAK), and polyarteritis nodosa (PAN).^[2] These conditions are often autoimmune in nature, though exact causes remain largely unknown, and they can occur idiopathically or secondary to infections like hepatitis B or other immune disorders.^[3] The most common type, giant cell arteritis, primarily affects individuals over 50 years old, with a higher incidence in women and those of Northern European descent, occurring at a rate of about 10-25 cases per 100,000 people annually in that age group.^[2] It typically involves the cranial arteries, such as the temporal artery, leading to symptoms like severe headaches, scalp tenderness, jaw pain during chewing (jaw claudication), and visual disturbances that can progress to blindness if untreated.^[4] Takayasu arteritis, in contrast, predominantly impacts younger women under 40, especially in Asia, with an incidence of 1-2 per million, targeting the aorta and its major branches to cause fatigue, limb pain (claudication), hypertension, and reduced pulses in the arms.^[2] Polyarteritis nodosa affects medium-sized arteries throughout the body, often in adults aged 40-60, and is historically associated with hepatitis B virus in up to 30% of cases, though this rate has declined to about 7% following widespread HBV vaccination and blood product screening, manifesting as fever, weight loss, nerve damage (mononeuritis multiplex), skin lesions, and organ involvement like kidney or gastrointestinal infarction.^[2]^[5] Diagnosis of arteritis typically involves blood tests showing elevated inflammatory markers like erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), imaging such as ultrasound, CT angiography, or MRI, and sometimes biopsy of affected arteries.^[3] Treatment focuses on suppressing inflammation with high-dose corticosteroids like prednisone, often combined with immunosuppressive agents such as methotrexate or biologic drugs like tocilizumab for GCA, while addressing underlying causes (e.g., antiviral therapy for hepatitis-associated PAN).^[6] Early intervention is critical to prevent complications like stroke, aortic rupture, or irreversible vision loss, with prognosis varying by type—GCA responding well to therapy in most cases, while TAK may require lifelong monitoring for vascular stenosis.^[2]

Definition and Classification

Definition

Arteritis is a vascular disorder characterized by inflammation of the walls of arteries, which can lead to thickening, narrowing, or occlusion of the arterial lumen, thereby reducing blood flow to affected organs and tissues.^[7] This inflammatory process primarily targets the arterial vasculature, distinguishing it from phlebitis, which involves inflammation of the veins, and from broader vasculitis, a condition encompassing inflammation of all types of blood vessels including veins and capillaries.^[8]^[9] The condition was first described in the 19th century, with early reports linking arterial inflammation to infectious causes such as syphilis and tuberculosis.^[10] A notable advancement came in 1932 when Bayard T. Horton and colleagues coined the term "temporal arteritis" to describe an undescribed form of arteritis affecting the temporal vessels, marking a key step in recognizing noninfectious variants.^[11] Arteries consist of three primary layers: the intima (innermost endothelial lining), the media (middle layer of smooth muscle and elastic tissue), and the adventitia (outer connective tissue layer). In arteritis, inflammation typically involves the media but can extend transmurally to affect all layers, resulting in edema, cellular infiltration, and fibrotic changes that compromise vessel integrity and patency.^[12]^[13]

Classification

Arteritis is classified primarily according to the size of the affected vessels, as outlined in the 2012 Revised International Chapel Hill Consensus Conference (CHCC) nomenclature for vasculitides.^[14] Large-vessel arteritis involves the aorta and its major branches, such as the subclavian and carotid arteries.^[15] Medium-vessel arteritis predominantly affects the main visceral arteries and their branches, including renal and mesenteric arteries.^[15] Small-vessel arteritis, which targets arterioles, capillaries, and venules, is less commonly considered a pure form of arteritis due to its frequent involvement of non-arterial structures.^[15] The CHCC framework categorizes arteritis within the broader spectrum of vasculitis, emphasizing predominant vessel involvement while acknowledging that any vessel size may be affected to varying degrees.^[14] Under this system, large-vessel vasculitis includes giant cell arteritis (GCA), characterized by granulomatous inflammation typically in individuals over 50 years, and Takayasu arteritis, a granulomatous arteritis affecting the aorta and branches with onset usually before age 50.^[15] Medium-vessel vasculitis encompasses polyarteritis nodosa (PAN), a necrotizing arteritis of medium or small arteries without glomerulonephritis or antineutrophil cytoplasmic antibody (ANCA) association, and Kawasaki disease, an arteritis linked to mucocutaneous lymph node syndrome that often involves coronary arteries in children.^[15] Infectious arteritis represents a distinct category outside the primary autoimmune vasculitides, caused by direct microbial invasion of arterial walls.^[16] Examples include syphilitic aortitis due to Treponema pallidum, which leads to medial necrosis and aneurysmal dilation of the ascending aorta.^[16] Arteritis is differentiated from ANCA-associated vasculitides, which primarily affect small vessels and may secondarily involve arteries but are defined by necrotizing inflammation with few immune deposits and ANCA positivity.^[15]

Epidemiology

Incidence and Prevalence

Arteritis encompasses several types, with giant cell arteritis (GCA) and Takayasu arteritis being among the most prominent large-vessel variants, alongside medium-vessel involvement in polyarteritis nodosa (PAN). The global pooled incidence of GCA is approximately 10 cases per 100,000 individuals annually among those aged 50 years and older, with estimates ranging up to 25 cases per 100,000 in high-incidence regions such as Northern Europe.^[17]^[18] In contrast, Takayasu arteritis is considerably rarer, with an annual incidence of 0.4-2.6 cases per million population worldwide.^[19] Polyarteritis nodosa has an annual incidence of 0.9–8 cases per million in European countries and a prevalence of around 31 per million, primarily affecting adults without strong geographic variation but associated with hepatitis B in up to 30% of cases.^[20] Prevalence of GCA shows marked geographic variation, with higher rates in Northern Europe and North America; for instance, it reaches up to 200-300 per 100,000 among women over 50 in these regions.^[21] ^[22] Takayasu arteritis exhibits greater prevalence in Asia, estimated at 30-40 per million in populations such as those in Japan and India, compared to 3-13 per million in Europe.^[23] ^[19] Reported cases of arteritis, particularly GCA, have increased since the early 2000s, attributable to the adoption of advanced imaging techniques like ultrasound and MRI, which enhance detection of extracranial involvement.^[21] Recent studies from the 2020s indicate stable underlying incidence rates but improved identification through these modalities, leading to earlier diagnoses in high-resource settings.^[24] Underdiagnosis remains a challenge for arteritis, especially in low-resource settings where limited access to imaging and specialized care results in prolonged diagnostic delays—often months to years for Takayasu arteritis—and significant data gaps in regions like Africa, South America, and parts of Asia.^[25] ^[26] These factors contribute to underreporting, with nonspecific symptoms exacerbating missed cases in resource-constrained environments.^[27]

Risk Factors and Demographics

Giant cell arteritis (GCA), the most common form of systemic arteritis in older adults, predominantly affects individuals over the age of 50, with the highest incidence occurring between 70 and 79 years and a mean age of onset around 75 years.^[12] The disease is rare before age 50, and incidence rates increase steadily with advancing age.^[28] GCA demonstrates a female predominance, with a female-to-male ratio of approximately 3:1, as evidenced by incidence rates of 17.8 per 100,000 in females compared to 8.2 per 100,000 in males in population-based studies.^[29]^[30] Genetic factors play a role in GCA susceptibility, particularly associations with HLA-DRB104 alleles, including HLA-DRB10401 and HLA-DRB1*0404, which are more prevalent in patients of Northern European descent.^[12] These genetic markers contribute to disease risk, with familial aggregation observed in some cases.^[31] Ethnically, GCA incidence is highest among individuals of Scandinavian or Northern European ancestry, with lower rates reported in African American, Asian, and Hispanic populations.^[12] Environmental triggers, such as smoking, are linked to increased risk, with cohort studies showing odds ratios of 1.18 to 1.19 for current smokers compared to non-smokers.^[32] Possible infectious triggers, including reactivation of cytomegalovirus, have been implicated in immunosenescence-related pathogenesis.^[28] Takayasu arteritis, in contrast, primarily impacts younger adults, with most cases diagnosed between 40 and 50 years of age, though onset often occurs under 40.^[33] It exhibits a marked female predominance, with a female-to-male ratio as high as 9:1.^[33] While specific genetic associations are less well-defined, human leukocyte antigen linkages have been speculated but not conclusively established.^[33] Ethnically, Takayasu arteritis shows higher incidence in Asian populations, where it is more prevalent than in Western countries, and in individuals of Mexican or Hispanic descent.^[33] For instance, studies in diverse cohorts report elevated rates among Asians (up to 15.8% of cases) and Hispanics (10.5%).^[34] Environmental factors, including potential infectious exposures, have been proposed as triggers, though evidence remains correlative rather than causal.^[35]

Pathophysiology

Etiology

Arteritis refers to inflammation of arterial walls, with etiologies broadly categorized into idiopathic (primary) forms driven by autoimmune mechanisms and secondary forms triggered by identifiable factors such as infections or systemic diseases. In primary vasculitides like giant cell arteritis (GCA) and Takayasu arteritis, the precise initiating events remain elusive, but immune dysregulation plays a central role, leading to aberrant T-cell and cytokine responses without a discernible external trigger.^[12]^[29]^[33]^[36] Infectious agents can directly invade arterial walls or provoke an inflammatory response, resulting in secondary arteritis. Bacterial infections, such as those caused by Staphylococcus aureus, often manifest as mycotic arteritis, where septic embolization leads to focal arterial weakening and aneurysm formation, particularly in the aorta or peripheral vessels.^[37]^[38] Viral pathogens, including varicella-zoster virus (VZV), have been proposed in some studies to contribute to GCA exacerbations, with VZV DNA and antigens detected in temporal artery biopsies of affected patients, though this remains controversial and not supported by all research, suggesting possible reactivation as a trigger for disease flares.^[39]^[40]^[41] Spirochetal infections, notably tertiary syphilis caused by Treponema pallidum, classically produce aortitis through chronic endarteritis obliterans, affecting the vasa vasorum and leading to aortic dilation or aneurysm over 10-30 years post-infection.^[42]^[43] Secondary arteritis may also arise in the context of systemic autoimmune diseases or iatrogenic factors. For instance, rheumatoid arthritis (RA) is associated with rheumatoid vasculitis, a medium- and small-vessel arteritis that develops in less than 1% of patients with rheumatoid arthritis, though rates have declined with improved treatments, characterized by immune complex deposition and necrotizing inflammation in arterial walls.^[44]^[45] Drug-induced forms, such as minocycline-associated vasculitis, mimic polyarteritis nodosa and often present with cutaneous or systemic involvement, including neuropathy, after prolonged exposure (typically >1 year), resolving upon drug discontinuation.^[46]^[47]^[48] Genetic factors contribute to susceptibility across arteritis subtypes, particularly in idiopathic forms. Polymorphisms in the PTPN22 gene, such as the R620W variant (rs2476601), increase risk for GCA by impairing T-cell receptor signaling and enhancing autoreactivity, with meta-analyses confirming an odds ratio of approximately 1.7 in European populations.^[49]^[50]^[51] Genome-wide association studies (GWAS) as of 2024 have identified additional loci, including three novel ones for GCA beyond HLA-DRB1 alleles and IL12B variants, and confirmation of key factors like HLA-B*52 for Takayasu arteritis, underscoring shared genetic architecture with other autoimmune diseases and highlighting polygenic contributions to immune dysregulation in GCA and Takayasu arteritis.^[52]^[53]^[54]^[55]

Inflammatory Mechanisms

Arteritis involves immune-mediated inflammatory processes that target the arterial wall, primarily driven by adaptive and innate immune responses. In giant cell arteritis (GCA), dendritic cells in the arterial adventitia activate CD4+ T lymphocytes, polarizing them into Th1 and Th17 subsets; Th1 cells produce interferon-gamma to activate macrophages, while Th17 cells secrete IL-17, promoting further cytokine release from macrophages and fibroblasts.^[56] Macrophages and T-cells infiltrate all layers of the vessel wall, leading to granulomatous inflammation characterized by multinucleated giant cells formed from fused macrophages, which represent a chronic delayed-type hypersensitivity reaction.^[57] In Takayasu arteritis (TA), similar granulomatous inflammation occurs at the medio-adventitial junction, involving lymphocytes, plasma cells, and histiocytes.^[58] Cytokine storms amplify these pathways, with adventitial macrophages producing pro-inflammatory cytokines such as IL-1, IL-6, and TNF-alpha, which sustain T-cell differentiation and macrophage activation.^[56] IL-6 specifically drives Th17 polarization and is overexpressed in inflamed arteries, contributing to systemic inflammation, while IL-17A levels correlate with disease activity and response to therapy.^[53] In GCA, Th1 cells induced by IL-12 and IL-18 release IFN-γ, fostering chronic inflammation resistant to initial treatments.^[59] These cytokines also activate endothelial cells, upregulating adhesion molecules like VCAM-1 and ICAM-1 to facilitate leukocyte recruitment.^[60] Vascular remodeling ensues from this infiltration, involving intimal hyperplasia through smooth muscle cell proliferation and migration, often mediated by endothelin-1, leading to luminal stenosis.^[59] Medial destruction fragments the internal elastic lamina, while adventitial fibrosis thickens the outer layer, potentially resulting in aneurysms if the wall weakens excessively.^[56] In TA, chronic phases show replacement fibrosis in the media and loose fibrocellular intimal thickening, causing endothelial dysfunction and endarteritis obliterans.^[58] At the molecular level, matrix metalloproteinases (MMPs) play a crucial role in degrading the extracellular matrix; MMP-9 from monocytes enables T-cell invasion and is localized at sites of elastic lamina degeneration, while MMP-2 contributes to overall remodeling.^[59] Endothelial activation further promotes this by expressing adhesion molecules that tether inflammatory cells.^[60] The disease progresses from acute granulomatous inflammation to chronic fibrosis through overlapping histological stages. In GCA, early acute phases feature transmural infiltration, evolving to adventitia-limited or vasa vasorum involvement, with skip lesions—non-contiguous inflamed segments—observed in 8–26% of cases, explaining patchy disease distribution.^[53] In TA, progression includes active granulomatous, chronic mononuclear, and healing fibrotic phases, with persistent low-grade inflammation driving long-term vascular damage.^[58] This model highlights a shift from immune-driven injury to inappropriate tissue repair, perpetuating stenosis or aneurysms.^[57]

Signs and Symptoms

General Presentation

Arteritis manifests with systemic and vascular symptoms that vary by type, including large-vessel forms such as giant cell arteritis (GCA) and Takayasu arteritis (TAK), as well as medium-vessel forms like polyarteritis nodosa (PAN). Commonly, patients experience systemic symptoms reflecting widespread inflammation, including fever, fatigue, weight loss, and malaise, which occur in up to 50% of patients, particularly in GCA.^[17] Such constitutional features often precede or accompany more localized vascular involvement, contributing to the overall clinical picture of active disease.^[61] Vascular symptoms are hallmark signs across arteritis types, with headache—especially a new-onset temporal headache in GCA—affecting approximately 75% of cases.^[62] Patients may also experience claudication, such as pain in the limbs during exertion in TAK or jaw claudication during chewing in GCA, alongside pulsatile tenderness or reduced pulses over inflamed arteries.^[63] Organ-specific manifestations arise from ischemia in affected territories, including vision disturbances like transient monocular blindness (amaurosis fugax) in up to 30% of GCA patients and limb ischemia leading to pain or weakness.^[62] In PAN, symptoms often include mononeuritis multiplex (peripheral nerve damage affecting multiple nerves), skin lesions such as livedo reticularis or purpura, abdominal pain from mesenteric ischemia, and renal involvement leading to hypertension or infarction. These features reflect the medium-vessel involvement throughout the body.^[64]^[65] The onset of symptoms in arteritis varies by etiology and type. In GCA, presentation is typically subacute, evolving over weeks to months, though abrupt worsening can occur.^[66] Infectious forms of arteritis, such as those secondary to bacterial or viral pathogens, often exhibit a more acute onset with rapid progression of local pain, swelling, and systemic inflammatory signs.^[67]

Acute Complications

One of the most dreaded acute complications of giant cell arteritis (GCA) is permanent visual loss, primarily resulting from anterior ischemic optic neuropathy (AION). In untreated cases, approximately 15-20% of patients develop vision loss, often sudden and irreversible, affecting one or both eyes due to ischemia of the optic nerve head from inflammation of the posterior ciliary arteries.^[68] This complication accounts for at least 85% of all instances of vision impairment in GCA, with the risk of progression to the contralateral eye reaching 25-50% within the first week if not promptly managed.^[66] Permanent blindness can occur in up to 20% of affected individuals, underscoring the urgency of early intervention to preserve sight.^[69] In Takayasu arteritis, acute aortic emergencies such as aneurysm rupture or dissection pose significant life-threatening risks, particularly in patients with aneurysmal involvement. Aneurysms develop in 10-30% of cases, with the ascending aorta and aortic arch being common sites due to chronic granulomatous inflammation leading to vessel wall weakening.^[70] Rupture occurs in approximately 3.7% of those with aortic aneurysms, while dissection affects about 3.5%, often presenting with severe chest pain, hemodynamic instability, and high mortality if untreated.^[71] These events are more frequent in younger patients with prolonged disease duration exceeding five years, where the risk of aneurysm formation increases fourfold.^[72] Stroke and myocardial infarction represent critical cerebrovascular and cardiovascular complications in arteritis, driven by involvement of the carotid arteries or coronary vessels. In GCA, the incidence of stroke ranges from 2-7%, typically occurring within the first month of symptom onset and manifesting as ischemic infarcts in the vertebrobasilar or carotid territories due to arterial stenosis or embolism.^[73] Similarly, myocardial infarction risk is elevated, with an incidence of about 2.4% in biopsy-proven cases, often classified as type 2 infarction secondary to systemic inflammation and demand ischemia rather than primary coronary occlusion.^[74] These events contribute to heightened cardiovascular morbidity, with GCA patients facing 1.6- to 1.8-fold higher odds compared to age-matched controls.^[75] Less common but severe acute complications include scalp necrosis and gangrene from profound ischemia. In GCA, scalp necrosis arises in rare instances (less than 1% of cases) due to occlusion of the superficial temporal artery branches, leading to localized tissue death and ulceration, frequently co-occurring with visual loss in up to 79% of reported patients.^[76] In Takayasu arteritis, gangrene of the limbs develops from critical limb ischemia secondary to subclavian or femoral artery occlusion, though it remains infrequent owing to collateral vessel formation; case reports highlight its occurrence in advanced, untreated disease with absent pulses and claudication.^[77]^[78] In PAN, acute complications can include bowel perforation, myocardial infarction, or renal failure from vascular occlusion. These ischemic sequelae emphasize the potential for rapid decompensation in untreated arteritis.^[65]

Diagnosis

Clinical Assessment

Clinical assessment of arteritis begins with a detailed history and physical examination to identify suggestive features, particularly in common forms such as giant cell arteritis (GCA).^[12] History taking focuses on the onset and duration of headaches, which are often new-onset, unilateral, and temporal in location, affecting approximately 66% of patients with GCA.^[12] Inquiry into visual symptoms is crucial, including transient blurring, diplopia, or amaurosis fugax-like episodes, reported in 20-30% of cases.^[12] Additionally, clinicians should explore associations with polymyalgia rheumatica, manifesting as proximal muscle pain and stiffness, which co-occurs in 40-60% of GCA patients.^[12] Jaw claudication, fatigue during mastication, and constitutional symptoms like fever or weight loss further support suspicion.^[12] The physical examination targets vascular signs of arterial inflammation. Palpation of the temporal arteries may reveal absent or diminished pulses, tenderness, or nodularity in up to 50% of GCA cases.^[12] Scalp tenderness, especially over the temporal region, is a classic finding elicited by gentle traction of hair.^[12] Auscultation for bruits over the carotid, subclavian, or axillary arteries can indicate large-vessel involvement, while assessment of peripheral pulses may show asymmetry or diminution in the upper extremities.^[12] Diagnostic scoring systems aid in stratifying risk during initial evaluation. The 1990 American College of Rheumatology (ACR) criteria for GCA classification include age at onset greater than or equal to 50 years, new headache, temporal artery abnormality (tenderness or reduced pulsation), elevated erythrocyte sedimentation rate, and arterial biopsy showing vasculitis with giant cells; fulfillment of three or more criteria yields high specificity (91.2%) and sensitivity (93.5%).^[79] Red flags warranting urgent evaluation include sudden or profound vision loss, which signals ischemic optic neuropathy and occurs in about 8-10% of untreated GCA patients, necessitating immediate specialist referral to prevent permanent blindness.^[12] For Takayasu arteritis (TAK), clinical assessment emphasizes absent or diminished pulses ("pulseless disease"), bruits over large vessels, blood pressure discrepancies between arms, and symptoms like fatigue or claudication in young women.^[33] Polyarteritis nodosa (PAN) presents with systemic symptoms (fever, weight loss), mononeuritis multiplex, and abdominal pain; hepatitis B testing is key due to association in ~30% of cases.^[80]

Laboratory and Imaging Tests

Laboratory tests play a crucial role in supporting the diagnosis of arteritis, particularly in giant cell arteritis (GCA) and Takayasu arteritis, by identifying inflammatory markers and excluding other vasculitides. The erythrocyte sedimentation rate (ESR) is elevated above 50 mm/hr in more than 90% of GCA cases, often exceeding 100 mm/hr, though it may be normal in 4-20% of patients and thus does not rule out the condition.^[12]^[81] C-reactive protein (CRP) levels are also markedly raised, with sensitivity of 86-100% for detecting inflammation in GCA.^[82] Anemia of chronic disease is common, reflecting systemic inflammation and cytokine effects on erythropoiesis. In primary arteritides like GCA and Takayasu, antineutrophil cytoplasmic antibodies (ANCA) are typically negative, helping differentiate them from ANCA-associated vasculitides. For PAN, ESR and CRP are often elevated but less consistently than in GCA; hepatitis B serology is essential.^[80] Non-invasive imaging modalities provide confirmatory evidence of vascular inflammation, with ultrasound recommended as the first-line test for suspected cranial GCA. High-resolution ultrasound detects the "halo sign," characterized by homogeneous, hypoechoic circumferential wall thickening exceeding 1 mm around the temporal or axillary arteries, with a pooled sensitivity of 69% and specificity of 91% for GCA diagnosis.^[83] Active inflammatory walls lack calcifications, distinguishing them from atherosclerotic changes. For large-vessel involvement in GCA or Takayasu arteritis, magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) are preferred, revealing wall thickening, edema, and contrast enhancement with sensitivity of 80% and specificity of 90% in cranial arteries for GCA, and serving as the primary modality for Takayasu due to its ability to assess multi-segmental disease without radiation.^[84] Positron emission tomography-computed tomography (PET-CT) using 18F-fluorodeoxyglucose (FDG) is useful for detecting active large-vessel inflammation as of 2025, showing increased uptake in affected arteries with sensitivity around 80% and specificity of 90% for GCA, and is particularly useful for extracranial disease or when ultrasound is inconclusive.^[24] While ultrasound offers high specificity for GCA, its utility is lower in Takayasu arteritis, where MRI provides better comprehensive vessel assessment. For PAN, conventional angiography is key, demonstrating saccular microaneurysms or occlusions in medium arteries with high sensitivity (~90%), often in renal or mesenteric vessels.^[80] These tests build on clinical suspicion to guide diagnosis, with imaging increasingly favored over laboratory markers alone for specificity in large-vessel arteritis.

Histopathological Confirmation

Histopathological confirmation remains the gold standard for diagnosing arteritis, particularly through biopsy of affected arterial segments, which provides definitive evidence of vascular inflammation. For giant cell arteritis (GCA), the most common form affecting medium-sized arteries, temporal artery biopsy is the procedure of choice. This involves surgical excision of a 5 cm segment of the superficial temporal artery, typically performed unilaterally under local anesthesia, with bilateral sampling reserved for cases where initial results are negative but clinical suspicion remains high.^[85] The biopsy site is identified using Doppler ultrasound to ensure vessel patency, and the segment is ligated and sent for immediate histopathological analysis to minimize delays in treatment initiation.^[86] Risks associated with the procedure are low, with complication rates of 1-2%, primarily including wound infection, hematoma, and transient facial nerve injury.^[85] Microscopic examination reveals characteristic features that distinguish arteritis subtypes. In GCA, the hallmark is granulomatous inflammation involving the full thickness of the arterial wall (panarteritis), with mononuclear cell infiltrates, including lymphocytes and macrophages, often accompanied by multinucleated giant cells at the intima-media junction; however, giant cells are observed in only about 50% of positive biopsies.^[62] Disruption of the internal elastic lamina, intimal hyperplasia, and adventitial fibrosis are also common. In contrast, Takayasu arteritis, which primarily affects the aorta and its major branches, shows a predominantly lymphocytic infiltrate with plasma cells and macrophages in the media and adventitia during the active phase, potentially progressing to granulomatous changes with giant cells or fibrosis in chronic stages; biopsies for Takayasu are less frequently performed due to the deeper vessel locations but may involve subclavian or aortic sampling when feasible.^[87] For PAN, biopsy of involved tissues (e.g., sural nerve, skin, muscle, or kidney) demonstrates focal segmental necrotizing vasculitis of medium-sized arteries with fibrinoid necrosis and mixed inflammatory infiltrate, but without granulomatous features or ANCA positivity.^[80] The diagnostic yield of biopsy is influenced by the focal nature of the disease, with skip lesions—areas of normal tissue interspersed with inflammation—necessitating adequate sampling length to achieve sensitivity of 77-90% in patients with active, untreated disease.^[12] Shorter segments increase false-negative rates, which can reach 10-20% even with optimal technique. Recent advances in histopathological evaluation include immunohistochemistry to characterize the inflammatory infiltrate, such as CD3 staining for T-lymphocytes and CD68 for macrophages, enhancing detection in subtle or treated cases where routine histology may be equivocal.^[88] Molecular markers like ALK1 are typically absent, helping to differentiate arteritis from mimics such as inflammatory myofibroblastic tumors.^[89] These techniques complement imaging findings by providing tissue-level confirmation of the inflammatory process.

Management

Pharmacological Therapies

Pharmacological therapies for arteritis primarily aim to suppress inflammation and prevent complications, with treatments tailored to the specific type such as giant cell arteritis (GCA), Kawasaki disease, or infectious forms. Corticosteroids form the cornerstone of therapy for most non-infectious vasculitides, while immunosuppressants serve as adjunctive agents to reduce steroid dependence and relapse risk. For infectious arteritis, antimicrobial agents are essential. High-dose corticosteroids, typically prednisone at 40-60 mg/day orally, are the first-line treatment for GCA and should be initiated promptly upon suspicion to halt disease progression. In cases of GCA with threatened or recent vision loss, intravenous methylprednisolone at 1000 mg daily for 3-5 days is recommended to achieve rapid suppression before transitioning to oral therapy. Therapy is tapered gradually over 12-24 months based on clinical response, with monitoring for relapse. Relapse rates in GCA range from 40-60% during tapering, often necessitating prolonged low-dose maintenance (e.g., 5-10 mg/day prednisone) to sustain remission. Adjunctive immunosuppressants are used in steroid-refractory or relapsing cases, particularly in GCA. Methotrexate (10-25 mg/week orally) reduces relapse risk by approximately 40-50% compared to corticosteroids alone and facilitates steroid tapering, though its glucocorticoid-sparing effect is modest. Tocilizumab, an interleukin-6 inhibitor, was FDA-approved in 2017 for GCA and significantly lowers relapse rates; in clinical trials, it achieved sustained remission in 56% of patients at 52 weeks versus 14-18% with placebo, representing a hazard ratio for flares of 0.23-0.28. It is administered subcutaneously (162 mg weekly or every other week) alongside a rapid steroid taper. Upadacitinib, a Janus kinase inhibitor, was FDA-approved in May 2025 for the treatment of adults with GCA and can be used as an adjunct to glucocorticoids to achieve and maintain disease remission while facilitating glucocorticoid taper.^[90] Type-specific therapies address unique aspects of arteritis variants. In Kawasaki disease, high-dose aspirin (80-100 mg/kg/day divided every 6 hours during fever) combined with intravenous immunoglobulin reduces the incidence of coronary artery aneurysms from approximately 25% to 5%, followed by low-dose aspirin (3-5 mg/kg/day) for antiplatelet effects until inflammation resolves.^[91] For polyarteritis nodosa, high-dose glucocorticoids are used for mild disease, with the addition of cyclophosphamide for severe, organ-threatening manifestations; in hepatitis B-associated cases, antiviral therapy combined with plasma exchange and limited glucocorticoids is recommended.^[92] For infectious arteritis, such as bacterial aortitis, intravenous antibiotics (e.g., broad-spectrum coverage with vancomycin and piperacillin-tazobactam, tailored by culture) for at least 6 weeks are critical to eradicate the pathogen and avert rupture.

Surgical and Supportive Interventions

Surgical interventions for arteritis are typically reserved for cases where severe vascular complications, such as critical stenoses or aneurysms, lead to ischemia or life-threatening risks, particularly in large-vessel vasculitides like Takayasu arteritis (TA) and giant cell arteritis (GCA). In TA, bypass grafting is indicated for significant stenoses, such as subclavian artery involvement causing arm claudication or renovascular hypertension, and is ideally performed during periods of disease remission to minimize perioperative complications. Aneurysm repair, often via graft replacement, is recommended for expanding or symptomatic aortic or branch aneurysms to prevent rupture. In GCA, surgical options are less common but may include revascularization for extracranial large-vessel stenoses or aortic aneurysmorrhaphy when medical therapy fails to control progression. Common procedures include bypass grafting using synthetic or autologous materials for stenotic lesions, with endarterectomy being rare due to high rates of restenosis and poor long-term patency in inflammatory arteriopathies. For large-vessel disease, percutaneous transluminal angioplasty (PTA) with or without stenting offers a less invasive alternative, achieving initial technical success rates of 80-90% in TA, though restenosis can occur in up to 20-30% of cases within 1-2 years, often necessitating repeat interventions. These procedures complement the pharmacological backbone of immunosuppression by addressing mechanical obstructions once inflammation is controlled. Supportive care plays a crucial role in managing symptoms and preventing flares in arteritis patients. Pain management for headaches in GCA or limb discomfort in TA involves nonsteroidal anti-inflammatory drugs (NSAIDs) or acetaminophen, used cautiously alongside glucocorticoids to avoid exacerbating gastrointestinal risks. Physical therapy, including supervised aerobic and resistance exercises, is beneficial for patients with claudication, improving functional capacity and reducing fatigue without increasing cardiovascular strain. Vaccination against preventable infections, such as influenza and pneumococcus, is advised for immunosuppressed individuals to mitigate flare triggers, with non-live vaccines preferred. A multidisciplinary approach is essential for optimal outcomes, involving coordination among rheumatologists for disease monitoring, ophthalmologists to address visual complications in GCA, and vascular surgeons for procedural planning and follow-up. This integrated care model enhances decision-making for interventions and supports holistic patient management.

Prognosis and Prevention

Long-term Outcomes

Long-term outcomes in arteritis vary by subtype, with giant cell arteritis (GCA) generally showing favorable survival but frequent relapses, while Takayasu arteritis (TA) is often more chronic with higher risks of vascular complications. Polyarteritis nodosa (PAN) has good prognosis with treatment, with 5-year survival rates exceeding 80-90%, though relapses occur in 20-40% of cases, often related to organ involvement.^[80]^[93] In GCA, early initiation of glucocorticoid therapy achieves remission rates of 70-80% in the majority of patients, though sustained drug-free remission remains uncommon, occurring in only about 21% after an average of 3 years.^[94]^[95] For TA, approximately 60% of patients experience chronic disease activity, with relapses occurring in approximately 40-60% within 5 years of diagnosis, often leading to progressive vascular damage.^[96]^[97] Survival rates are relatively high across subtypes when managed promptly, but complications influence prognosis. The 5-year survival rate for GCA exceeds 90%, with one study reporting 94.6%, approaching that of the general population despite initial disease-related risks.^[98] In TA, the 5-year survival rate is around 90-94%, though it declines to 80-89% at 10-15 years, primarily due to heart failure and vascular events, which contribute to approximately 20% of long-term mortality in severe cases.^[99]^[100]^[101] For PAN, untreated survival is poor (less than 20% at 5 years), but treatment improves it to over 80%.^[93] Relapses significantly affect long-term management, particularly in the absence of biologic therapies. In GCA, 40-50% of patients experience relapse within 5 years, with a cumulative rate of 46.6%, often necessitating prolonged immunosuppression.^[102] Relapse rates are even higher without biologics like tocilizumab, exceeding 50% despite extended treatment.^[103] For TA, relapses occur in up to 50% within 10 years, frequently tied to vascular complications that worsen prognosis.^[97] Chronic effects, especially from long-term glucocorticoid use, impair quality of life across arteritis subtypes. Steroid-related side effects, including osteoporosis, affect up to 30% of patients, leading to increased fracture risk and reduced bone health that persists post-remission.^[104]^[105] These complications, alongside cardiovascular and metabolic issues, contribute to diminished daily functioning and higher morbidity in 40-70% of long-term survivors.^[106]^[107]

Preventive Strategies

Primary prevention of arteritis focuses on modifiable risk factors to reduce incidence, particularly for giant cell arteritis (GCA). Smoking is an established risk factor for GCA, with meta-analyses indicating that current smokers have approximately 18% higher odds of developing the disease compared to non-smokers (OR 1.18), and ever-smokers 19% higher odds (OR 1.19); thus, smoking cessation is recommended to mitigate this elevated risk.^[32] In regions with high tuberculosis prevalence, treating latent tuberculosis infection (LTBI) is crucial, as LTBI occurs in up to 50% of Takayasu arteritis (TAK) patients and can reactivate under immunosuppressive therapy, potentially exacerbating vasculitic inflammation.^[108] For PAN, screening and antiviral treatment for hepatitis B, present in up to 30% of cases, can prevent onset.^[3] Secondary prevention emphasizes early detection in high-risk populations. Patients over 50 with polymyalgia rheumatica (PMR), which shares inflammatory pathways with GCA and is associated with 40-50% of cases (and can precede, coincide with, or follow GCA), should undergo regular monitoring of erythrocyte sedimentation rate (ESR); elevations above 30-40 mm/hour warrant prompt evaluation for arteritis to enable timely intervention.^[109] To prevent disease flares in established arteritis, management strategies include controlled tapering of glucocorticoids, as abrupt reductions increase relapse risk by up to 50% in GCA; guidelines advocate gradual dose decreases over months while monitoring symptoms and inflammatory markers.^[110] For TAK specifically, annual vascular imaging, such as computed tomography angiography or magnetic resonance angiography, is advised to detect subclinical progression and guide adjustments in therapy.^[111] Emerging preventive approaches include genetic counseling for individuals with familial arteritis histories, given evidence of heritable susceptibility loci in GCA and rare familial clustering in TAK, allowing for risk assessment and family screening.^[112] Pilot studies from 2022-2023 suggest statins may reduce vascular inflammation in PMR and GCA patients at risk, potentially lowering disease activity through pleiotropic anti-inflammatory effects, though larger trials are needed to confirm efficacy.^[113]

References

[1]
Vasculitis - Symptoms and causes - Mayo Clinic
Feb 5, 2025 · Vasculitis involves swelling and irritation, called inflammation, of blood vessels. The inflammation can cause the walls of the blood vessels to thicken.Giant cell arteritis · Diagnosis and treatment · Takayasu's arteritis · Buerger disease<|control11|><|separator|>
[2]
Spectrum of Large and Medium Vessel Vasculitis in Adults
Sep 27, 2022 · The Primary Vasculitides · Giant Cell Arteritis · Takayasu Arteritis · Polyarteritis Nodosa · Kawasaki Disease · Anti-neutrophil Cytoplasmic Antibody ...
[3]
Vasculitis - American College of Rheumatology
There are many types of vasculitis, such as giant cell arteritis (GCA), Kawasaki disease, microscopic polyangiitis (MPA), granulomatosis with polyangiitis ...
[4]
Giant cell arteritis - Symptoms and causes - Mayo Clinic
Sep 21, 2022 · Giant cell arteritis is an inflammation of the lining of your arteries. Most often, it affects the arteries in your head, especially those in your temples.
[5]
Giant cell arteritis - Diagnosis and treatment - Mayo Clinic
Sep 21, 2022 · Head pain and tenderness are the most common symptoms of giant cell arteritis, an inflammation of the lining of your arteries.
[6]
Arteritis: Causes, Types & Diagnosis - Healthline
Mar 15, 2016 · Arteritis refers to inflammation of your arteries that damages your blood vessel walls and reduces blood flow to your organs.
[7]
Types of Vasculitis
“Angiitis” and “Arteritis” are both synonyms for vasculitis, literally meaning “inflammation within blood vessels” or “inflammation in arteries.” Because there ...Giant Cell Arteritis · Takayasu's Arteritis · Rheumatoid Vasculitis · Behcet's Disease
[8]
Vasculitis and Thrombophlebitis - Medscape Reference
May 10, 2023 · Vasculitis is defined as inflammation of blood vessels. It may result in vessel wall thickening, stenosis, and occlusion with subsequent ischemia.
[9]
Arteritis | Causes, Symptoms, Diagnosis, Treatment - Britannica
Arteritis, inflammation of an artery or arteries. Arteritis may occur in a number of diseases, including syphilis, tuberculosis, pancreatic disease, ...
[10]
Bayard Horton's Clinicopathological Description of Giant ... - PubMed
He reported these two patients in 1932 as 'an undescribed form of arteritis of the temporal vessels'. He was the first to describe jaw claudication. He saw a ...
[11]
Giant Cell Arteritis (Temporal Arteritis) - StatPearls - NCBI Bookshelf
May 2, 2024 · Giant cell arteritis (GCA) is a chronic inflammatory vasculitis that predominantly affects large- and medium-sized arteries in individuals older than 50.
[12]
Arteritis - an overview | ScienceDirect Topics
Arteritis. Vasculitis in general terms describes inflammation and necrosis of blood vessels and refers predominantly to arterial disease. Classification of ...
[13]
2012 Revised International Chapel Hill Consensus Conference ...
Oct 8, 2012 · 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. J. C. Jennette,. Corresponding Author.
[14]
Overview of the 2012 Revised International Chapel Hill Consensus ...
This overview summarizes the 2012 CHCC and points out the changes compared to the 1994 CHCC. Notable changes include the introduction of new terms.
[15]
Aortitis | Circulation - American Heart Association Journals
Jun 10, 2008 · The classification of aortitis broadly includes underlying rheumatologic and infectious diseases, along with isolated aortitis (Table 1). The ...
[16]
A new era for giant cell arteritis - PMC - PubMed Central - NIH
Oct 3, 2019 · The positive news is that fast track pathways, imaging techniques and new therapies are emerging for routine management of GCA.
[17]
Takayasu Arteritis: JACC Focus Seminar 3/4
Jan 3, 2023 · A recent systematic review found a global prevalence of 3.2 to 40.0 cases per million and an annual incidence of 0.4 to 2.6 cases per million.
[18]
Incidence and prevalence of giant cell arteritis and polymyalgia ...
This systematic literature review (SLR) aims to summarize current evidence regarding the global incidence and prevalence of GCA and PMR.
[19]
Contemporary Prevalence Estimates for Giant Cell Arteritis ... - NIH
The prevalence rate of GCA among women was 304 (95%CI: 229–375) and among men was 91 (95%CI: 46–156) per 100,000 population aged ≥50 years. Prevalence rates of ...
[20]
Takayasu arteritis: Prevalence and clinical presentation in Switzerland
Jun 18, 2021 · The reported prevalence varies between 4.7-33/1'000'000 in Western European countries and 30-40/1'000'000 in Asian populations [2–7].
[21]
Giant cell arteritis: reviewing the advancing diagnostics and ...
Feb 14, 2023 · Temporal arteries can be spared in 40% of patients with LV-GCA, risking misdiagnosis when relied upon in isolation for diagnosis [98]. LV-GCA ...Temporal Artery Examination · Rapid Access Gca Pathways · Management Of Gca
[22]
Global epidemiology of vasculitis | Nature Reviews Rheumatology
Dec 1, 2021 · Fig. 1: Global incidence of giant cell arteritis. a | Giant cell arteritis occurs most frequently in populations of Northern European ancestry.
[23]
Advances in Takayasu arteritis: An Asia Pacific perspective - Frontiers
Takayasu Arteritis (TA) is a rare form of chronic granulomatous large vessel vasculitis that is more common in Asia compared to other parts of the world.
[24]
A systematic review and meta-analysis of the incidence rate of ...
Nov 3, 2021 · Conclusions: TAK is an extremely rare disease. It affects women more commonly than men. There is considerable variation in the incidence rate ...
[25]
Giant cell arteritis: immune and vascular aging as disease risk factors
The single strongest risk factor is age, with incidence steadily rising in the seventh to eighth decades of life [9,10]. The disease essentially does not exist ...Missing: demographics | Show results with:demographics
[26]
Giant Cell Arteritis (Temporal Arteritis) - Medscape Reference
Sep 22, 2025 · Giant cell arteritis (GCA), or temporal arteritis, is a systemic inflammatory vasculitis of unknown etiology that occurs in older persons.Missing: types | Show results with:types
[27]
Epidemiology of biopsy-confirmed giant cell arteritis in southern ...
Mar 20, 2021 · The average annual incidence 1997–2019 was 13.3 (95% CI: 12.6, 14.0) per 100 000 inhabitants aged ≥50 years and was higher in females (17.8; 95% ...Patients And Methods · Results · Incidence Of Tab+ GcaMissing: global | Show results with:global
[28]
Genetic component of giant cell arteritis - Oxford Academic
Jul 10, 2013 · GCA has been consistently associated with MHC class II molecules (i.e. HLA-DR3, HLA-DR4, HLA-DR5 and HLA-DRB1) in many independent studies, ...Abstract · Introduction · HLA associations · Associations with genes...
[29]
Smoking as a risk factor for giant cell arteritis - PubMed
The GCA cohort showed an association with current smoking with an odds ratio of 1.18 (95% CI, 1.01-1.38). Conclusion: Our study demonstrated a statistically ...
[30]
Takayasu Arteritis - StatPearls - NCBI Bookshelf
Takayasu arteritis, aka pulseless disease, is a systemic inflammatory condition which leads to damage of the medium and large arteries and their branches.Takayasu Arteritis · Pathophysiology · Treatment / Management
[31]
Demographic, Clinical, and Radiologic Characteristics of a Cohort of ...
The median age at diagnosis was 29.8 years (interquartile range, 23-38), and 61.4% were Caucasian, 15.8% Asian, 10.5% Hispanic and 8.8% African American (Table ...Missing: ethnic | Show results with:ethnic
[32]
Environmental Triggers for Vasculitis - PubMed - NIH
Many data support infectious triggers in some vasculitides, whereas other studies have identified noninfectious triggers, such as airborne pollutants, silica, ...
[33]
Takayasu's arteritis - Symptoms & causes - Mayo Clinic
Sep 10, 2025 · This rare disease that harms the aorta, the large artery that carries blood from the heart to the rest of the body.
[34]
Mycotic Aneurysm - StatPearls - NCBI Bookshelf
Apr 3, 2025 · A mycotic aneurysm is an infection of the blood vessel wall caused by bacterial, fungal, or viral pathogens.
[35]
Spectrum of Large- and Medium-Vessel Vasculitis in Adults
Aug 3, 2022 · Bacterial infections Staphylococci and streptococci are the most common gram-positive pathogens leading to MVV or LVV, usually of the aorta and ...
[36]
Varicella zoster virus and giant cell arteritis - PMC - NIH
Primary VZV infection causes varicella (chickenpox), after which virus becomes latent in ganglionic neurons along the entire neuraxis. Decades later, as VZV- ...Missing: flares | Show results with:flares
[37]
Varicella Zoster Virus Commonly Found in Giant Cell Artery Biopsies
Jun 16, 2015 · Varicella-zoster virus infection was studied in temporal artery ... VZV antigen was found in 61/82 (74%) GCA-positive temporal arteries ...
[38]
Syphilitic Aortitis - an overview | ScienceDirect Topics
Syphilitic aortitis is defined as an inflammation of the aorta that occurs as a manifestation of tertiary syphilis, typically 10 to 30 years post-infection, ...
[39]
Syphilitic aortitis and its complications in the modern era - PubMed
Dec 16, 2016 · Introduction: Aortitis is a well-recognized manifestation of the tertiary stage of syphilis. Evidence acquisition: Although often regarded ...
[40]
Rheumatoid Vasculitis: Symptoms & Treatment - Cleveland Clinic
Rheumatoid vasculitis is a type of vasculitis that affects people who have rheumatoid arthritis (RA) for a long time. It causes inflammation in your blood
[41]
Rheumatoid Vasculitis
Feb 5, 2024 · When RA affects the blood vessels, it is called rheumatoid vasculitis. Vasculitis is a family of rare disorders characterized by inflammation of ...
[42]
Lymphocytic Thrombophilic Arteritis Induced by Minocycline - PMC
The authors present the first case of a newly termed entity, lymphocytic thrombophilic arteritis, which coincides with the use of minocycline. Although the ...
[43]
Keys for Recognizing Drug-Induced Vasculitis - Consult QD
May 31, 2023 · In contrast to primary PAN, patients with minocycline-induced medium vessel vasculitis are commonly found to have positive ANCA antibodies.
[44]
Palindromic Minocycline-Induced Vasculitis With Dual-Positivity for ...
Sep 11, 2025 · Long-term usage of minocycline can result in the development of various autoimmune conditions, including vasculitis.Missing: arteritis | Show results with:arteritis
[45]
Identification of the PTPN22 functional variant R620W as ... - NIH
Our data indicate, for the first time, an important role for PTPN22 in the genetic susceptibility of GCA. The combined analysis of the four independent cohorts ...Missing: 2023 | Show results with:2023
[46]
PTPN22 R620W minor allele is a genetic risk factor for giant cell ...
Apr 7, 2016 · PTPN22 may be an archetypal autoimmunity gene. · In this study we have estimated the frequency of the PTPN22 rs2476601 (R620W) variant in ...
[47]
Identification of the PTPN22 functional variant R620W as ... - PubMed
Our results suggest that the PTPN22 polymorphism rs2476601/R620W plays an important role in the genetic risk to GCA.
[48]
Risk loci involved in giant cell arteritis susceptibility: a genome-wide ...
May 8, 2024 · Giant cell arteritis is an immune-mediated inflammatory disease with a strong genetic component that remains poorly understood. Our incomplete ...
[49]
Giant cell arteritis: reviewing the advancing diagnostics and ... - Nature
Feb 14, 2023 · The aim of this review was to present the latest advances in GCA that have the potential to influence routine clinical practice.
[50]
Pathogenesis of giant cell arteritis: More than just an inflammatory ...
Macrophages in the adventitia produce pro-inflammatory cytokines such as IL-1, IL-6 and tumor necrosis factor alpha. These cytokines promote arterial wall and ...
[51]
Current Insights into Tissue Injury of Giant Cell Arteritis - PubMed
Feb 29, 2024 · The tissue inflammatory bulk is expressed as acute or chronic delayed-type hypersensitivity reactions, the latter being apparent by giant cell formation.
[52]
Pathophysiology, Diagnosis, and Management of Takayasu Arteritis
Jul 29, 2023 · TA is characterized by autoimmune-mediated inflammation, vascular remodeling, and endothelial dysfunction. The disease progresses through three ...
[53]
https://www.nature.com/articles/s41433-023-02433-y
[54]
Giant cell arteritis: pathogenic mechanisms and new potential ...
Nov 28, 2017 · Significant advances in the understanding of the genetic and cellular mechanisms in GCA are leading to identification of potential pathogenic targets.
[55]
Large-Vessel Vasculitis - Rheumatology Advisor
Nov 16, 2022 · Large-vessel vasculitis is an umbrella term for diseases that affect the large arteries. The two main types are giant cell arteritis and Takayasu arteritis.Missing: organ- | Show results with:organ-
[56]
Giant cell arteritis | Radiology Reference Article - Radiopaedia.org
Oct 22, 2025 · Clinical presentation · headache (75%) · systemic symptoms (e.g. fever, fatigue, weight loss) · jaw claudication (30%) · arterial symptoms and signs.Temporal arteritis · Presentation · Case · Sonographic halo sign...<|control11|><|separator|>
[57]
Takayasu's Arteritis : Johns Hopkins Vasculitis Center
viral, bacterial, or other — occurring in a person with ...First Description · Classic symptoms of Takayasu... · How is Takayasu's Arteritis...
[58]
Clinical manifestations of giant cell arteritis - UpToDate
Systemic symptoms associated with GCA are frequent and include fever, fatigue, and weight loss. Fevers occur in up ...
[59]
Infectious Cavernous Sinus Thrombosis Presenting as Temporal ...
In the earlier phase of the disease, CST often presents with nonspecific headache, fever, lethargy, and altered mentation. The average time between clinical ...
[60]
A typical case of giant cell arteritis with vision loss due to delayed ...
If left untreated, 15%‐20% of patients experience vision loss; however, the rate of vision loss can be reduced to 1% with glucocorticoid treatment.
[61]
Recurrent Vision Loss in a Patient with Giant Cell Arteritis while on ...
Apr 1, 2024 · Permanent visual loss occurs in up to 20% of patients, consisting of partial or complete loss in one or both eyes. ... Diagnosis relies on ...
[62]
A Multifaceted Case of Takayasu Arteritis With Aneurysmal ...
Oct 31, 2025 · The identification of a 4.0 cm infrarenal aortic aneurysm is a critical finding, as aneurysms occur in 10%–30% of TA patients and carry a ...
[63]
AORTIC INVOLVEMENT IN TAKAYASU ARTERITIS: A NATIONAL ...
Oct 18, 2022 · Among TAK patients with AA, 20 (3.7%) and 19(3.5%) had an aortic rupture and aortic dissection, respectively. ... proportion of traditional ...
[64]
Takayasu Arteritis with Dyslipidemia Increases Risk of Aneurysm
Oct 1, 2019 · The risk of aneurysm was 4.2-fold higher in patients with disease duration >5 years. Persistent inflammation of the aortic vessel wall is ...<|separator|>
[65]
Acute Lateral Medullary Infarct due to Giant Cell Arteritis | Stroke
Sep 9, 2019 · Among patients with GCA, ischemic strokes occur in ≈2% to 7% depending on the study, with strokes most frequently occurring in the ...
[66]
Myocardial infarction during giant cell arteritis: A cohort study
Feb 17, 2021 · GCA-related myocardial infarction (MI) is a rare event (2.4% of cases). · GCA-related MI are mainly type 2 MI rather than type 1 MI due to ...
[67]
Abstract 4144705: Risk of Cardiovascular Disease in Giant Cell ...
Nov 11, 2024 · The endpoint included any cardiovascular events, myocardial infarction (MI), coronary artery disease (CAD), aortic aneurysm/dissection ...
[68]
Scalp Necrosis in Giant Cell Arteritis and Review of the Literature
Nineteen (79%) had other serious complications of GCA: visual loss in 16, gangrene of the tongue in four and nasal septum necrosis in one. The mean interval ...
[69]
Gangrene in Takayasu's arteritis: a report of two cases and review of ...
The vascular inflammation in TA results in stenoses of affected vessels. Usually this is a slow process with good collateral circulation; only rarely does ...
[70]
Takayasu Arteritis - Musculoskeletal and Connective Tissue Disorders
Etiology of Takayasu Arteritis The cause of Takayasu arteritis is unknown. Cell-mediated immune mechanisms may be involved.
[71]
The American College of Rheumatology 1990 criteria for ... - PubMed
For the traditional format classification, 5 criteria were selected: age greater than or equal to 50 years at disease onset, new onset of localized headache, ...
[72]
Temporal Artery Biopsy - StatPearls - NCBI Bookshelf - NIH
May 27, 2023 · Biopsy of the superficial temporal artery is currently the gold standard for diagnosing giant cell arteritis. This activity reviews the ...Continuing Education Activity · Indications · Preparation · Technique or Treatment
[73]
Temporal artery biopsy: A technical guide and review of its ...
Temporal artery biopsy (TAB) is a surgical procedure that enables the histological diagnosis of giant cell arteritis (GCA).
[74]
Pathology of Takayasu arteritis: A brief review - PMC - PubMed Central
The pathological features vary with the stage of the disease and include granulomatous inflammation, elasophagia, adventitial and medial fibrosis, vessel ...
[75]
Temporal artery biopsy in giant cell arteritis - Frontiers
The typical histological findings in GCA are often characterized by granulomatous inflammation with infiltration of mononuclear cells with or without the ...
[76]
CD3 immunohistochemistry is helpful in the diagnosis of giant cell ...
CD3 staining performed routinely on temporal artery biopsy specimens might improve the sensitivity of temporal artery biopsy in patients with biopsy-negative ...
[77]
ALK - Pathology Outlines
Mar 7, 2022 · Anaplastic lymphoma kinase gene is at 2p23; protein is called ALK1, CD246; Membrane spanning tyrosine kinase receptor, member of insulin ...Missing: arteritis | Show results with:arteritis
[78]
Few Patients Achieve Sustained Drug-Free Remission in Giant Cell ...
Jan 17, 2025 · Sustained drug-free remission (SDFR) is rare in giant cell arteritis (GCA), achieved by roughly 21% of patients after an average of 3 years.
[79]
Measuring treatment outcomes and change in disease activity in ...
Remission was reported in 73% of RCTs and 42% of LOS; It was predominantly defined as the combination of clinical and laboratory components. One LOS reported ...
[80]
Predictors of relapse in Takayasu arteritis - ScienceDirect
Previous studies have reported that 50–96% of TAK patients experienced relapses within 5 years of diagnosis [1,3,4]. Active disease increases vascular damage ...
[81]
Long-Term Outcomes and Prognostic Factors of Complications in ...
Sep 19, 2017 · This nationwide study shows that 50% of patients with TA will relapse and experience a vascular complication ≤10 years from diagnosis.
[82]
Clinical Manifestations and Prognosis of Giant Cell Arteritis - PubMed
In 45 (28.5%) out of 158 patients, a stable remission was achieved as a result of GC monotherapy; in 120 (75.9%) patients, long-term maintenance therapy with ...Missing: outcomes | Show results with:outcomes
[83]
Increased mortality rate in Takayasu arteritis is largely driven by ...
Among 224 patients with TAK (159 females, mean follow-up duration 44.36 months), survival at 1, 2, 5 and 10 years was 97.34%, 96.05%, 93.93% and 89.23%, ...Missing: vascular events
[84]
OP0070 ONSET TO DIAGNOSIS TIME PREDICTS SURVIVAL RATE ...
5-year survival rate was 92%; 10-year survival rate was 90%; 15-year survival rate was 80%. The median term of survival was 34 [20 - 41] years. 31 deaths (18 ...Missing: mortality vascular
[85]
Takayasu arteritis: advanced understanding is leading to new horizons
Apr 4, 2018 · Daily activities are compromised in 74%, while mortality remains up to 5% at 10 years [1] and as high as 27% in the most severely affected [5].
[86]
Epidemiology and Predictors of Relapse in Giant Cell Arteritis
One-year, 2-year and 5-year CRRs were 32.0% [95% CI 22.4 – 43.6%], 44.3% [95% CI 30.5 – 59.1%], and 46.6% [95% CI 27.2 – 67.1%], respectively. The duration of ...<|separator|>
[87]
Relapse Risk and Safety of Long-Term Tocilizumab Use Among ...
Nov 1, 2025 · TCZ is well tolerated in GCA, with low rates of discontinuation for AESIs. However, relapse occurred in > 50% despite median treatment > 12 months.
[88]
Rapid Tapering Reduces Steroid-toxicity in GCA - Steritas
Apr 22, 2024 · Glucocorticoid adverse events such as osteoporosis, hyperglycaemia/diabetes mellitus and infections affect up to 85% of GCA patients, leading to ...
[89]
The Impact of High Dose Glucocorticoids on Bone Health and ...
Feb 16, 2022 · This review will outline the known mechanism of bone loss in vasculitis and will summarize factors attributing to fracture risk in different types of ...
[90]
Patient perceptions of health-related quality of life in giant cell arteritis
Feb 2, 2021 · Adverse effects are common including psychological symptoms, cardiovascular disease, osteoporosis and diabetes [7–9].
[91]
Steroid-Sparing Agents in Giant Cell Arteritis
These suggested that 40-48% of GCA patients require additional immunosuppressive agents to achieve remission and to taper their corticosteroid intake.
[92]
Prevalence of tuberculosis infection among patients with Takayasu ...
Dec 18, 2023 · Moreover, the study revealed that patients with TAK displayed a high prevalence of latent TB infection (LTBI) at 50.01% (95% CI 31.25–68.77%) ...
[93]
Polymyalgia Rheumatica and Giant Cell Arteritis - AAFP
Systemic symptoms: weight loss, fever, fatigue, anorexia. Peripheral pulse disturbances. LR− = negative likelihood ratio; LR+ = positive likelihood ratio ...
[94]
Trial of Tocilizumab in Giant-Cell Arteritis
Jul 27, 2017 · In conclusion, tocilizumab combined with a 26-week prednisone taper was superior to either a 26-week or 52-week prednisone taper plus placebo ...
[95]
2018 Update of the EULAR recommendations for the management ...
We recommend that a suspected diagnosis of LVV should be confirmed by imaging or histology. High dose glucocorticoid therapy (40–60 mg/day prednisone-equivalent) ...
[96]
Risk loci involved in giant cell arteritis susceptibility: a genome-wide ...
Our results indicate that genetic profiling could be useful for predicting risk of giant cell arteritis and represent a further step towards the implementation ...Missing: counseling | Show results with:counseling
[97]
Do statins decrease vascular inflammation in patients at risk for ...
Apr 18, 2023 · Our preliminary results suggest that statins may exert a potential protective role on vascular inflammation in patients with PMR and GCA. Statin ...Missing: Takayasu pilot 2022-2024