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Current Rheumatology Reports Nov 2020To provide an overview of mimickers of large vessel vasculitis (LVV), by the main presenting manifestation, i.e., systemic, vascular, and cranial manifestations. (Review)
Review
PURPOSE OF REVIEW
To provide an overview of mimickers of large vessel vasculitis (LVV), by the main presenting manifestation, i.e., systemic, vascular, and cranial manifestations.
RECENT FINDINGS
The main differential diagnoses in patients with giant cell arteritis (GCA) and Takayasu arteritis (TAK) presenting with systemic manifestations (i.e., fever, anorexia, weight loss, night sweats, arthralgia/myalgia, and/or increased inflammatory indexes) are neoplastic, infectious, or other inflammatory conditions. In patients with vascular manifestations (such as peripheral ischemia, vascular stenoses, or aneurysms), atherosclerosis and non-inflammatory vascular diseases should be excluded. In those presenting with predominant cranial symptoms (i.e., temporal headache, jaw claudication, scalp tenderness, transient or permanent vision loss), other causes of headache, cerebrovascular accidents, optic neuropathy, and neuromuscular syndromes need to be considered. The diagnosis of LVV maybe challenging, especially when patients present with atypical or incomplete clinical forms. In these cases, a multidisciplinary approach is strongly recommended.
Topics: Atherosclerosis; Diagnosis, Differential; Giant Cell Arteritis; Humans; Stroke; Takayasu Arteritis; Vasculitis
PubMed: 33159612
DOI: 10.1007/s11926-020-00965-w -
Polish Archives of Internal Medicine Jun 2022Large vessel vasculitis (LVV), including Takayasu arteritis (TAK) and giant cell arteritis (GCA), causes granulomatous vascular inflammation mainly in large vessels, and...
Large vessel vasculitis (LVV), including Takayasu arteritis (TAK) and giant cell arteritis (GCA), causes granulomatous vascular inflammation mainly in large vessels, and is the most common primary vasculitis in adults. Vascular inflammation may evoke many clinical features including vision impairment, stroke, limb ischemia, and aortic aneurysms. The best way to diagnose LVV is to combine medical history, physical examination, various laboratory tests, and imaging modalities. Progress in imaging modalities facilitated early diagnosis and follow‑up of the disease activity. Conventional angiography is no longer the gold standard for the diagnosis of TAK. Similarly, temporal artery biopsy is no longer the only tool for diagnosing cranial GCA. In selected cases, color Doppler ultrasound may be used for this purpose. Despite some similarities, TAK and GCA differ in many aspects and they are different diseases. They also have different clinical subtypes. The presence of aortitis does not always implicate the diagnosis of TAK or GCA; infectious aortitis, as well as noninfectious aortitis associated with other autoimmune rheumatic diseases should be excluded. Treatment of LVV includes glucocorticoids (GCs), conventional immunosuppressive agents, and biological drugs. Tumor necrosis factor inhibitors are ineffective in GCA but effective in TAK. On the other hand, tocilizumab may be used to treat both diseases. Promising targeted therapies evaluated in ongoing clinical trials include, for example, anti‑IL‑12/23 (ustekinumab), anti‑IL‑17 (secukinumab), anti‑IL‑1 (anakinra), anti‑IL‑23 (guselkumab), anti‑cytotoxic T‑lymphocyte antigen 4 (abatacept), Janus kinase inhibitors (tofacitinib and upadacitinib), anti‑granulocyte / macrophage colony‑stimulating factor (mavrilimumab), and endothelin receptor (bosentan) therapies.
Topics: Adult; Aortitis; Giant Cell Arteritis; Glucocorticoids; Humans; Inflammation; Takayasu Arteritis
PubMed: 35699647
DOI: 10.20452/pamw.16272 -
Archives of Pathology & Laboratory... Jan 2017Ischemic optic neuropathy (ION) describes a state of hypoxic injury of the optic nerve. Clinically, ION is divided into anterior and posterior forms defined by the... (Review)
Review
Ischemic optic neuropathy (ION) describes a state of hypoxic injury of the optic nerve. Clinically, ION is divided into anterior and posterior forms defined by the presence or absence of optic disc swelling, respectively. It is further classified as arteritic when secondary to vasculitis, and nonarteritic when not. The site of vascular occlusion for anterior ION from giant cell arteritis is the short posterior ciliary arteries, but mechanical vascular obstruction does not play a role in most nonarteritic cases. Histologically, ION is characterized by axon and glial necrosis, edema, and a sparse mononuclear response. Like other ischemic injuries, the morphologic alternations in the nerve are time dependent. A variant of ION called cavernous degeneration (of Schnabel) features large cystic spaces filled with mucin. Several conditions can histologically mimic cavernous degeneration of the optic nerve. The scarcity of cases of ION examined histologically has contributed to an incomplete understanding of its pathogenesis.
Topics: Arteritis; Diagnosis, Differential; Humans; Optic Disk; Optic Nerve; Optic Neuropathy, Ischemic
PubMed: 28029908
DOI: 10.5858/arpa.2016-0027-RS -
Rheumatology International Feb 2019There are no universally accepted diagnostic criteria for large-vessel vasculitides (LVV), including giant cell arteritis (GCA) and Takayasu arteritis (TAK). Currently,... (Review)
Review
There are no universally accepted diagnostic criteria for large-vessel vasculitides (LVV), including giant cell arteritis (GCA) and Takayasu arteritis (TAK). Currently, available classification criteria cannot be used for the diagnosis of GCA and TAK. Early diagnosis of these two diseases is quite challenging in clinical practice and may be accomplished only by combining the patient symptoms, physical examination findings, blood test results, imaging findings, and biopsy results, if available. Awareness of red flags which lead the clinician to investigate TAK in a young patient with persistent systemic inflammation is helpful for the early diagnosis. It should be noted that clinical presentation may be highly variable in a subgroup of GCA patients with predominant large-vessel involvement (LVI) and without prominent cranial symptoms. Imaging modalities are especially helpful for the diagnosis of this subgroup. Differential diagnosis between older patients with TAK and this subgroup of GCA patients presenting with LVI may be difficult. Various pathologies may mimic LVV either by causing systemic inflammation and constitutional symptoms, or by causing lumen narrowing with or without aneurysm formation in the aorta and its branches. Differential diagnosis of aortitis is crucial. Infectious aortitis including mycotic aneurysms due to septicemia or endocarditis, as well as causes such as syphilis and mycobacterial infections should always be excluded. On the other hand, the presence of non-infectious aortitis is not unique for TAK and GCA. It should be noted that aortitis, other large-vessel involvement or both, may occasionally be seen in various other autoimmune pathologies including ANCA-positive vasculitides, Behçet's disease, ankylosing spondylitis, sarcoidosis, and Sjögren's syndrome. Besides, aortitis may be idiopathic and isolated. Atherosclerosis should always be considered in the differential diagnosis of LVV. Other pathologies which may mimic LVV include, but not limited to, congenital causes of aortic coarctation and middle aortic syndrome, immunoglobulin G4-related disease, and hereditary disorders of connective tissue such as Marfan syndrome and Ehler-Danlos syndrome.
Topics: Diagnosis, Differential; Early Diagnosis; Giant Cell Arteritis; Humans; Takayasu Arteritis
PubMed: 30221327
DOI: 10.1007/s00296-018-4157-3 -
Frontiers in Immunology 2022Vasculitis is an autoimmune vascular inflammation with an unknown etiology and causes vessel wall destruction. Depending on the size of the blood vessels, it is... (Review)
Review
Vasculitis is an autoimmune vascular inflammation with an unknown etiology and causes vessel wall destruction. Depending on the size of the blood vessels, it is classified as large, medium, and small vessel vasculitis. A wide variety of immune cells are involved in the pathogenesis of vasculitis. Among these immune cells, monocytes and macrophages are functionally characterized by their capacity for phagocytosis, antigen presentation, and cytokine/chemokine production. After a long debate, recent technological advances have revealed the cellular origin of tissue macrophages in the vessel wall. Tissue macrophages are mainly derived from embryonic progenitor cells under homeostatic conditions, whereas bone marrow-derived circulating monocytes are recruited under inflammatory conditions, and then differentiate into macrophages in the arterial wall. Such macrophages infiltrate into an otherwise immunoprotected vascular site, digest tissue matrix with abundant proteolytic enzymes, and further recruit inflammatory cells through cytokine/chemokine production. In this way, macrophages amplify the inflammatory cascade and eventually cause tissue destruction. Recent studies have also demonstrated that monocytes/macrophages can be divided into several subpopulations based on the cell surface markers and gene expression. In this review, the subpopulations of circulating monocytes and the ontogeny of tissue macrophages in the artery are discussed. We also update the immunopathology of large vessel vasculitis, with a special focus on giant cell arteritis, and outline how monocytes/macrophages participate in the disease process of vascular inflammation. Finally, we discuss limitations of the current research and provide future research perspectives, particularly in humans. Through these processes, we explore the possibility of therapeutic strategies targeting monocytes/macrophages in vasculitis.
Topics: Arteritis; Cytokines; Giant Cell Arteritis; Humans; Inflammation; Macrophages; Monocytes
PubMed: 35967455
DOI: 10.3389/fimmu.2022.859502 -
International Journal of Rheumatic... Feb 2016Childhood-onset Takayasu arteritis (c-TA) is a distinct subset affecting a wide age group, ranging from young infants to adolescents and it differs from adult TA in many... (Review)
Review
Childhood-onset Takayasu arteritis (c-TA) is a distinct subset affecting a wide age group, ranging from young infants to adolescents and it differs from adult TA in many aspects. There is scarcity of data on c-TA worldwide. The disease is classified using the European League Against Rheumatism/Pediatric Rheumatology International Trials Organization/Pediatric Rheumatology European Society criteria. The non-specific nature of presenting complaints and lack of appropriate biomarkers delay the early diagnosis of this illness and many children present with complications, which become irreversible once they set in. One of the largest cohorts of 40 children with c-TA from our center reports hypertension as the commonest presenting feature. Systemic symptoms like headache, fever and weight loss are also described. Assessment of disease in c-TA is done by correlating clinical features with raised inflammatory markers. Advanced imaging plays an important role in diagnosis. In c-TA, the role of magnetic resonance angiography is advocated, taking into consideration the enormous amount of radiation exposure with other modalities. Complications of c-TA include cardiovascular, pulmonary, neurological and those arising secondary to long-term steroid and immunosuppression therapy.
Topics: Adolescent; Age of Onset; Child; Child, Preschool; Diagnosis, Differential; Female; Genetic Predisposition to Disease; Humans; Infant; Male; Phenotype; Predictive Value of Tests; Risk Factors; Severity of Illness Index; Takayasu Arteritis; Treatment Outcome
PubMed: 26585174
DOI: 10.1111/1756-185X.12718 -
Deutsche Medizinische Wochenschrift... Nov 2021In recent years, clinically significant advances have been made in the management of giant cell arteritis and Takayasu arteritis. This concise review article highlights... (Review)
Review
In recent years, clinically significant advances have been made in the management of giant cell arteritis and Takayasu arteritis. This concise review article highlights important aspects of the diagnostic workup and imaging-based treatment surveillance of the large vessel vasculitides.
Topics: Aged; Arteries; Arteritis; Humans; Male; Middle Aged
PubMed: 34826836
DOI: 10.1055/a-1286-6663 -
Immunological Reviews Mar 2023The term "vasculitis" refers to a group of rare immune-mediated diseases characterized by the dysregulated immune system attacking blood vessels located in any organ of... (Review)
Review
The term "vasculitis" refers to a group of rare immune-mediated diseases characterized by the dysregulated immune system attacking blood vessels located in any organ of the body, including the skin, lungs, and kidneys. Vasculitides are classified according to the size of the vessel that is affected. Although this observation is not specific to small-, medium-, or large-vessel vasculitides, patients show a high circulating neutrophil-to-lymphocyte ratio, suggesting the direct or indirect involvement of neutrophils in these diseases. As first responders to infection or inflammation, neutrophils release cytotoxic mediators, including reactive oxygen species, proteases, and neutrophil extracellular traps. If not controlled, this dangerous arsenal can injure the vascular system, which acts as the main transport route for neutrophils, thereby amplifying the initial inflammatory stimulus and the recruitment of immune cells. This review highlights the ability of neutrophils to "set the tone" for immune cells and other cells in the vessel wall. Considering both their long-established and newly described roles, we extend their functions far beyond their direct host-damaging potential. We also review the roles of neutrophils in various types of primary vasculitis, including immune complex vasculitis, anti-neutrophil cytoplasmic antibody-associated vasculitis, polyarteritis nodosa, Kawasaki disease, giant cell arteritis, Takayasu arteritis, and Behçet's disease.
Topics: Humans; Polyarteritis Nodosa; Mucocutaneous Lymph Node Syndrome; Takayasu Arteritis; Inflammation; Skin
PubMed: 36408947
DOI: 10.1111/imr.13170 -
Rheumatology (Oxford, England) Jul 2016Takayasu arteritis (TAK) is a systemic granulomatous large-vessel vasculitis with a phenotype that overlaps with GCA and defined by the 1993 and 2012 Chapel Hill... (Review)
Review
Takayasu arteritis (TAK) is a systemic granulomatous large-vessel vasculitis with a phenotype that overlaps with GCA and defined by the 1993 and 2012 Chapel Hill Consensus Conference statements. However, the diagnosis of TAK is often delayed since TAK patients may be asymptomatic or have non-specific symptoms. Once a diagnosis is made, it is difficult to judge remission or recurrence since there are no reliable assessment tools. With the availability of newer agents, such as cytokine blockade, which are being evaluated in GCA, there is the potential for real advances in TAK patient management. Without reliable assessment tools it will be difficult to introduce newer agents in an organized way or to optimally benefit patients in the future. In this article we review the use and performance of disease indicators in TAK clinical trials as a basis for the further development of assessment tools for this disease.
Topics: Cytokines; Diagnosis, Differential; Giant Cell Arteritis; Humans; Outcome Assessment, Health Care; Phenotype; Symptom Assessment; Takayasu Arteritis
PubMed: 26472566
DOI: 10.1093/rheumatology/kev366 -
Heart (British Cardiac Society) Apr 2018Takayasu arteritis (TA) is a rare disease affecting chiefly young women, although it can affect both men and women and persons of many different ethnicities. TA carries... (Review)
Review
Takayasu arteritis (TA) is a rare disease affecting chiefly young women, although it can affect both men and women and persons of many different ethnicities. TA carries a high morbidity rate, but importantly, overall mortality has declined over time such that the 15-year survival rate has increased from 82.9% for patients diagnosed between 1957 and 1975 to 96.5% for those diagnosed from 1976 to 1990. Severity of presenting arterial complications and delay to diagnosis have also decreased over the past decade owing to advances in non-invasive diagnostic imaging and the development of medical therapies. Despite these advances, there still remain significant gaps in the diagnosis and management of these complex patients. These gaps encompass the basic, yet extremely complex, tasks of defining a universally accepted diagnostic criterion, accurate assessment of disease activity and development of clinically meaningful and accurate outcome measures to guide necessary clinical trials for the management of these complex patients.
Topics: Cardiac Imaging Techniques; Humans; Patient Care Management; Takayasu Arteritis
PubMed: 29175979
DOI: 10.1136/heartjnl-2016-310848