"Takayasu is a T-cell–mediated panarteritis with genetic predisposition and an unknown environmental or infectious trigger. The earliest histological change is adventitial and medial infiltration by lymphocytes, plasma cells, and macrophages, often forming granulomas with multinucleated giant cells. The inflammation begins around the vasa vasorum and progresses inward, ultimately involving all layers of the vessel wall. Involvement is patchy, with skip lesions and manifests as 21:"
"type I: the branches from aortic arch"
"type II:"
"IIa: the ascending aorta, the aortic arch and its branches"
"IIb: the ascending aorta, aortic arch and its branches and thoracic descending aorta"
"type III: the thoracic descending aorta, abdominal aorta and/or renal arteries"
"type IV: the abdominal aorta and/or the renal arteries"
"type V: combination of IIb and IV"
"increased FDG uptake on 18F-FDG PET CT is a sensitive marker of active arteritis which can precede anatomical changes"
"Familial clustering supports a genetic predisposition, and the strongest association is with HLA-B52, which also predisposes for more severe vascular damage 23. There are possible hormonal or sex-linked genetic influences and there is evidence for polyautoimmunity in 20%, which commonly manifests as coexisting spondyloarthritis-spectrum disorders (ankylosing spondylitis, inflammatory bowel disease, and psoriasis), Behçet disease, and others 24. Autoantibody positivity (e.g. ANA, ANCA, anticardiolipin, anti-beta-2 glycoprotein I), earlier disease onset, and refractory vasculitis are more common in this subgroup 25."
"Familial clustering supports a genetic predisposition, and the strongest association is with HLA-B52, which also predisposes for more severe vascular damage 23. There are possible hormonal or sex-linked genetic influences and there is evidence for polyautoimmunity in 20%, which commonly manifests as coexisting spondyloarthritis-spectrum disorders (ankylosing spondylitis, inflammatory bowel disease, and psoriasis), Behçet disease, and others 24. Autoantibody positivity (e.g. ANA, ANCA, anticardiolipin, anti-beta-2 glycoprotein I), earlier disease onset, and refractory vasculitis are more common in this subgroup 25."
"Familial clustering supports a genetic predisposition, and the strongest association is with HLA-B52, which also predisposes for more severe vascular damage 23. There are possible hormonal or sex-linked genetic influences and there is evidence for polyautoimmunity in 20%, which commonly manifests as coexisting spondyloarthritis-spectrum disorders (ankylosing spondylitis, inflammatory bowel disease, and psoriasis), Behçet disease, and others 24. Autoantibody positivity (e.g. ANA, ANCA, anticardiolipin, anti-beta-2 glycoprotein I), earlier disease onset, and refractory vasculitis are more common in this subgroup 25."
"The classification of Takayasu arteritis is based on the 2022 American College of Rheumatology (ACR) and European Alliance of Associations for Rheumatology (EULAR) criteria 18. The American College of Cardiology and American Heart Association diagnostic criteria differ in the detail 19."
"Corticosteroids are used for initial treatment followed by steroid-sparing agents, e.g. methotrexate, azathioprine, mycophenolate mofetil, leflunomide, cyclophosphamide, tocilizumab, and TNF-alpha inhibitors 14. The autoimmune subgroup are more likely to require biologic therapy due to refractory vasculitis 24."
"Corticosteroids are used for initial treatment followed by steroid-sparing agents, e.g. methotrexate, azathioprine, mycophenolate mofetil, leflunomide, cyclophosphamide, tocilizumab, and TNF-alpha inhibitors 14. The autoimmune subgroup are more likely to require biologic therapy due to refractory vasculitis 24."
Expected headings
"CT coronary angiography"
"Familial clustering supports a genetic predisposition, and the strongest association is with HLA-B52, which also predisposes for more severe vascular damage 23. There are possible hormonal or sex-linked genetic influences and there is evidence for polyautoimmunity in 20%, which commonly manifests as coexisting spondyloarthritis-spectrum disorders (ankylosing spondylitis, inflammatory bowel disease, and psoriasis), Behçet disease, and others 24. Autoantibody positivity (e.g. ANA, ANCA, anticardiolipin, anti-beta-2 glycoprotein I), earlier disease onset, and refractory vasculitis are more common in this subgroup 25."
"Early nonspecific constitutional symptoms (fever, malaise, weight loss, fatigue and night sweats) progress to vascular inflammation and arterial stenosis, occlusion or aneurysm formation, resulting in regional ischaemia:"
"Early nonspecific constitutional symptoms (fever, malaise, weight loss, fatigue and night sweats) progress to vascular inflammation and arterial stenosis, occlusion or aneurysm formation, resulting in regional ischaemia:"
"FDG-PET CT, MRI or CT with contrast medium are recommended and demonstrate important imaging features for diagnosis and assessment of disease activity and progress as follows 26,27:"
"long, smooth, homogeneous and moderately echogenic circumferential thickening of the arterial wall may be present"
"Treatment is with systemic steroids, immunosuppression and judicious use of angioplasty."
"stenosis, occlusion, and aneurysmal dilatation develop later and most commonly affect the thoracic and abdominal aorta, subclavian, carotid, renal, and pulmonary arteries"
"Corticosteroids are used for initial treatment followed by steroid-sparing agents, e.g. methotrexate, azathioprine, mycophenolate mofetil, leflunomide, cyclophosphamide, tocilizumab, and TNF-alpha inhibitors 14. The autoimmune subgroup are more likely to require biologic therapy due to refractory vasculitis 24."
"Prognosis is variable; approximately 50% experience relapses or vascular complications within 10 years of diagnosis. The autoimmunity subgroup experience earlier disease onset, higher disease activity, and more frequent relapses, often requiring biologic therapy for disease control 25."