Takayasu arteritis: advanced understanding is leading to new horizons

Abstract

Although outcomes in Takayasu arteritis (TAK) are improving, diagnosis is typically delayed and significant arterial injury accrues. While wider use of non-invasive imaging is impacting this, the onus remains with clinicians to consider a diagnosis of TAK earlier. Meanwhile, morbidity and mortality in TAK remains increased. Herein we review the current situation, outline recent advances and summarize remaining challenges. Understanding of disease pathogenesis remains poor. However, recent genetic data and identification of pathogenic cytokines may facilitate the search for biomarkers capable of distinguishing active and inactive disease, inflammatory and non-inflammatory arterial remodelling. Imaging is critical for TAK, and each modality has important strengths and limitations. Dependence upon CS therapy remains too high. However, the impact of combination immunosuppressive therapy is now recognized, biologic therapies are increasingly available and new agents offer promise. Multicentre clinical trials are now required, and these will depend upon development of defined clinical and imaging end-points.

Title: Human Serum/Plasma Glycoprotein Analysis by 1H-NMR, an Emerging Method of Inflammatory Assessment

“…Takayasu Arteritis (TA) TA is a rare, idiopathic systemic inflammatory disease affecting large arteries, including the aorta, its major branches, and the pulmonary arteries. Arterial inflammation is the core feature of the disease, variably associated with a systemic acute-phase response [193]. Novel biomarkers are required to distinguish inflammatory and non-inflammatory remodeling in Takayasu arteritis.…”

Isolated peripheral pulmonary stenosis in Takayasu arteritis

“…Like MRA, CTA can be used to detect areas of vessel thickening and to obtain a generalized survey of the major arteries and stenosis/dilation without the risks of arterial puncture of conventional angiography. Positron emission tomography scanning may provide valuable information about cellular activity within an inflamed arterial wall before morphologic changes appear on other imaging studies …”

“…Obstructive and aneurysmal arterial lesions often lead to end organ damage and ischemia in TA and therefor various surgical and interventional methods of revascularization have been studied and described. Evidence suggests that these procedures should be avoided in the active phase of the disease due to high restenosis rates and reserved for situations with critical organ ischemia such as myocardial or cerebral infarction or risk of aneurysm rupture …”

Complex interventions of abdominal aorta and its branches in children with Takayasu arteritis: Clinical experience from a tertiary care center in north‐west India

“…There is a faster progression of angiographic lesions, higher rate of restenosis, repeat intervention and poor long‐term outcome if intervention is performed during active disease . Hence, it is important to treat active TA disease by immunosuppressants such as glucocorticoids, methotrexate, MMF, azathioprine and anti‐tumor necrosis factor‐alpha (TNF‐α) agents prior to PTA . We used immunosuppressive therapy (prednisolone and methotrexate) to control active disease in three patients (cases 1, 2 and 5) prior to PTA, while the remaining three patients had inactive disease as assessed by inflammatory markers, that is ESR and CRP.…”