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Concept: Arteritis

169

Takayasu’s arteritis is an inflammatory arteriopathy which involves the aorta and its major branches, causing mainly stenosis of their lumen, though aneurysmal lesions can also occur. A young female with Takayasu’s disease presented to our hospital with acute lung oedema due to severe aortic insufficiency and ascending aorta dilatation. She had already undergone distal ascending aorta and hemiarch replacement due to Standford type A aortic dissection five years ago. The patient had also undergone reconstruction of abdominal arteries' stenoses with extraanatomical bypass. We performed a Bentall procedure with a valved conduit and implantation of the coronary ostia as buttons on the conduit. A mechanical valved graft was used instead of a bioprosthesis, due to possible early degradation of a bioprosthesis. The postoperative course was uneventful and the one year follow-up was normal. Valve-sparing aortic root replacement should be avoided in Takayasu’s arteritis due to high rate of recurrent regurgitation.

Concepts: Cardiology, Heart, Aortic aneurysm, Aortic dissection, Aorta, Ascending aorta, Takayasu's arteritis, Arteritis

168

Fever of unknown origin is a diagnostic challenge. Among its causes are of large caliber vessels vasculitis (LCVV), including Takayasu arteritis (TA) and giant cell arteritis (GCA). Early diagnosis is vital to prevent fibrosis of the vessel wall, and consequently, stenoses, aneurysms or occlusions. Imaging techniques can be of great help in recent years, highlighting the temporal artery through ultrasound, MRI and PET-CT.

Concepts: Blood vessel, Medical imaging, Positron emission tomography, Positron, Radiology, Giant cell arteritis, Takayasu's arteritis, Arteritis

29

A previously healthy 36-year-old Japanese woman presented with blood pressure that was difficult to control after an emergency cesarean section. CT revealed a disrupted abdominal aorta, severe stenosis of the renal arteries, and development of numerous collateral arteries.

Concepts: Kidney, Artery, Abdominal aorta, Renal artery, Caesarean section, Takayasu's arteritis, Arteritis, Angiology

23

Long-term outcome studies in Takayasu arteritis (TA) are few and limited by small sample size. In this study, we analysed the outcome of treatment in a large series of TA patients with a minimum follow-up period of ≥12 months by objective instruments.

Concepts: Medicine, Sample size, Hospital, Cardiology, India, Tamil Nadu, Takayasu's arteritis, Arteritis

23

Takayasu’s arteritis is known to cover the youngest age group in the etiology of secondary hypertension. The type 3 pattern is rare and involves the thoraco-abdominal aorta and its main abdominal branches. Here, we present the imaging results of a patient who had been followed up for systemic arterial hypertension and was diagnosed with Takayasu’s arteritis type 3 by CT and MR angiography following bilateral detection of a parvus-tardus Doppler signal modulation in the renal arteries. We discuss the differential diagnoses that should be considered when observing a tardus-parvus Doppler signal. © 2017 Wiley Periodicals, Inc. J Clin Ultrasound, 2017.

Concepts: Atherosclerosis, Medical terms, Cardiology, Blood pressure, Cardiovascular diseases, Takayasu's arteritis, Arteritis, Angiology

22

Takayasu arteritis (TA) is a giant cell arteritis usually affecting young women and characterized by inflammatory and ischemic signs of large vessel involvement, including extracranial cerebral arteries. The impact of stroke on TA prognosis has not been well evaluated.

Concepts: Cardiology, Blood vessel, Vasculitis, Cardiovascular diseases, Giant cell arteritis, Takayasu's arteritis, Arteritis, Angiology

6

Takayasu arteritis (TAK) is a large vessel vasculitis that rarely affects children. Data on childhood TAK are scarce. The aim of this study was to analyze the presenting features, course and outcome of children with TAK, compare efficacy of treatment regimens and identify high-risk factors for adverse outcome.

Concepts: Vasculitis, Takayasu's arteritis, Arteritis

0

The objective of the study was to investigate the long-term treatment effects and predictive factors for treatment response and disease relapse for Takayasu’s arteritis (TA). Eighty-one patients were recruited from the Department of Rheumatology, Zhongshan Hospital, Fudan University, between January 2009 and January 2015. The follow-up duration ranged from 6 to 36 months. Patients were divided into three groups: clinical remission (CR; n = 59); treatment-resistant (TR; n = 11); and disease relapse (DR; n = 11). Signs/symptoms, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and imaging items were recorded at baseline and at each visit. Kerr’s criteria, physician’s global assessment, and Indian Takayasu Clinical Activity Score (ITAS2010) were used to evaluate disease activity. Incipient disease was more common in CR patients compared with DR cases (69.49 vs. 36.36%, p = 0.05). Fewer patients aged < 40 years were in the CR group in comparison with the DR group (57.63 vs. 90.91%, p = 0.04). In TR patients, high CRP levels (63.74 vs. 23.73%, p = 0.01) and aortic arch involvement (70.00 vs. 24.14%, p < 0.01) were more common in comparison with CR cases. Patients with high CRP levels (> 25 mg/L) (OR = 1.61, p = 0.03) carried a higher risk for treatment resistance. Age > 40 years (OR = - 2.82, p = 0.03), incipient disease (OR = - 2.47, p = 0.01), and treatment with cyclophosphamide (OR = - 2.07, p = 0.03) and hydroxychloroquine (OR = - 1.91, p = 0.05) could prevent disease relapse. Patients with high CRP levels carry a high risk of treatment resistance. In patients with incipient disease, aged > 40 years, treatment with cyclophosphamide and hydroxychloroquine protects against disease relapse.

Concepts: Death, Red blood cell, Rheumatoid arthritis, C-reactive protein, Erythrocyte sedimentation rate, Takayasu's arteritis, Arteritis, Polymyalgia rheumatica

0

Juvenile-Takayasu arteritis (j-TA) is a difficult diagnosis and some patients develop uncommon manifestations and associated diseases that may contribute to the delayed diagnosis. Our aim was to identify the misdiagnoses, the associated diseases and the atypical manifestations observed in a j-TA Brazilian multicentre study. 71 children and adolescents who met the classification criteria for j-TA were included. The misdiagnoses, the associated diseases and the atypical manifestations were evaluated. 19 (26.8%) patients had misdiagnoses. The most common of them was aortic coarctation in six (8.4%) patients, followed by rheumatic fever in five (7.0%) and one patient presented with both former diagnoses. Limb pain (two patients), spondyloarthropathy, juvenile idiopathic arthritis (JIA), spinal arteriovenous malformation, polyarteritis nodosa (PAN) and fever of unknown origin (FUO) were other misdiagnoses. Patients who had misdiagnoses previously to j-TA diagnosis presented a trend to have a longer diagnosis delay. 11 (15.5%) patients had 14 TA-associated diseases, such as pulmonary tuberculosis (5 patients), rheumatic fever (2 patients), spondyloarthropathy, polyarticular JIA, Crohn’s disease, Prader-Willi disease, diabetes mellitus, Moyamoya and primary immunodeficiency. 7 (9.9%) patients presented 10 atypical manifestations, such as pyoderma gangrenosum, erythema nodosum, myositis, chorea, enthesitis, episcleritis, uveitis, hepatomegaly, splenomegaly and necrosis of extremities. Our study emphasizes the main misdiagnoses, associated diseases and atypical manifestations that occur in patients with j-TA and warns of the features that may alert paediatricians to this diagnosis, such as constitutional symptoms and elevated inflammatory markers.

Concepts: Infectious disease, Medical terms, Rheumatoid arthritis, Rheumatology, Sarcoidosis, Pyoderma gangrenosum, Polyarteritis nodosa, Arteritis

0

To assess the efficacy of tocilizumab in patients with Takayasu arteritis (TA).

Concepts: Takayasu's arteritis, Arteritis