Isolated peripheral pulmonary stenosis in Takayasu arteritis

Abstract

Takayasu arteritis is a chronic granulomatous vasculitis generally affecting large vessels including the thoracic aorta and its branches. Intimal proliferation subsequently causes segmental stenosis and occlusion, and frequently aneurysmal dilatation in the affected vessels. Although involvement of pulmonary arteries is not uncommon, isolated branch pulmonary stenosis is rarely encountered. Here, we report a patient presenting with isolated peripheral pulmonary stenosis as the primary manifestation of Takayasu arteritis. K E Y W O R D Smultimodality imaging, pulmonary stenosis, Takayasu arteritis

Percutaneous transluminal pulmonary angioplasty for Takayasu arteritis‐associated pulmonary hypertension: A single‐arm meta‐analysis

“…[32][33][34] The current meta-analysis integrating preliminary evidence suggests that PTPA can improve hemodynamics in patients with TA-PH. Although some similarities of imaging features such as stenosis and occlusion between TA-PH and CTEPH, 11,35,36 the pathological differences determine that the experience of PTPA treating CTEPH cannot be directly applied to TA-PH. | 565 vessels of TA-PH calls for different PTPA strategies.…”

Heart Failure in a Child

“…Isolated PA vasculitis secondary to TA is difficult to prove. The majority of cases are diagnosed on the basis of biopsy findings or retrospectively when systemic arteries are involved ( 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 ). Pulmonary vasculitis patients with pulmonary hypertension are at increased risk of early mortality ( 10 ).…”

“… Improvement in symptoms 3 y 2. Limited endarterectomy with steroids and azathioprine 25% reduction in PA pressure (mPAP 40 > 30) Improvement in 6-minute walk distance 3 mo 1 y Leibscher et al, 2017 ( 8 ) 56 Exertional dyspnea, syncope, and chest pain Diffuse stenosis of MPA and RPA Corticosteroids (3 mo) and azathioprine → relapsed in few months → methyl prednisolone (3 d) and methotrexate Improvement in symptoms but PA stenosis persisted 18 mo Alizadehasl et al, 2020 ( 9 ) 30 Progressive dyspnea and fatigue Severe stenosis of RPA and LPA Corticosteroids and azathioprine Improvement in symptoms and significant reduction of PA pressure (>50%) 3 y …”

Localized Forms of Vasculitis

“…Vasculitis restricted to the lower limbs, particularly the calves, has also been described [1,2] and patients with this variant of SOV may present AAV-, PAN-, or GCA-type lesions [126][127][128][129][130][131]. Rare cases of eosinophilic coronary monoarteritis [132][133][134] and isolated pulmonary arteritis, also named isolated pulmonary Takayasu's arteritis [135][136][137][138][139], have also been reported in the literature and may be associated with life-threatening disease manifestations.…”