Diagnosis and Assessment of Disease Activity in Takayasu Arteritis: A Childhood Case Illustrating the Challenge

Watson, Louise; Brogan, Paul A.; Peart, Ian; Landes, Caren; Barnes, Nik; Cleary, Gavin

doi:10.1155/2014/603171

Cited by 19 publications

(37 citation statements)

References 30 publications

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“…Untreated TA commonly leads to cardiovascular injuries, such as aneurysm formation, concentric arterial wall fibrosis and thrombotic complications [ 8 , 9 ]. Because large artery biopsies are usually infeasible and laboratory investigations are neither sensitive nor specific, the diagnosis of TA is based on clinical features, including a history compatible with systemic inflammation (with or without documentation of acute-phase response) and angiographic features compatible with LVV [ 7 , 8 ]. Angiography refers to either formal catheter, computed tomography angiography (CTA) or magnetic resonance angiography (MRA) [ 10 , 11 ].…”

Section: Introductionmentioning

confidence: 99%

Takayasu arteritis in childhood: retrospective experience from a tertiary referral centre in the United Kingdom

Eleftheriou¹,

Varnier²,

Doležalová³

et al. 2015

Arthritis Res Ther

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IntroductionTakayasu arteritis (TA) is an idiopathic large-vessel vasculitis affecting the aorta and its major branches. Although the disease rarely affects children, it does occur, even in infants. The objective of this study was to evaluate the clinical features, disease activity, treatment and outcome of childhood TA in a tertiary UK centre.MethodsWe analysed a retrospective case series of children fulfilling the TA classification criteria of the European League against Rheumatism, the Paediatric Rheumatology European Society and the Paediatric Rheumatology International Trials Organisation. Data regarding demographics, clinical features, treatments and outcomes were recorded. Descriptive statistics are expressed as median and range. Fisher’s exact test was used for group comparisons. The Paediatric Vasculitis Activity Score (PVAS), Paediatric Vasculitis Damage Index (PVDI), Disease Extent Index-Takayasu (DEI.Tak) and Indian Takayasu Arteritis Activity Score (ITAS2010) were calculated retrospectively.ResultsA total of 11 children (64% female) with age at diagnosis of 11.8 (1.3 to 17) years were identified over a 23-year period. The median time to diagnosis was 17 (0 to 132) months. The most common clinical features at presentation were arterial hypertension (72.7%), systemic features (36%) and cardiovascular (45%), neurological (36%), pulmonary (27%), skin (9%), renal (9%) and gastrointestinal (9%) involvement. At presentation, PVAS was 5/63 (1 to 13); DEI.Tak was 7/81 (2 to 12) and ITAS2010 was 9/57 (6 to 20). Treatment included corticosteroids (81.8%), combined with methotrexate in most cases (72.7%). Cyclophosphamide (36.4%) and biologic agents (45.5%) were reserved for severe and/or refractory cases. PVDI at latest follow-up was 5.5/72 (3 to 15). Mortality was 27%. Young age at disease onset (<5 years old) and permanent PVDI scores ≥3 were significantly associated with mortality risk (P = 0.024).ConclusionTA is a rare and potentially life-threatening large-vessel vasculitis. Improved awareness of TA is essential to secure a timely diagnosis. Although the evidence base for the treatment of TA in children is weak, we found that it is essential to treat it aggressively because our data emphasise that the mortality and morbidity in the paediatric population remains high.Electronic supplementary materialThe online version of this article (doi:10.1186/s13075-015-0545-1) contains supplementary material, which is available to authorized users.

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Section: Introductionmentioning

confidence: 99%

Takayasu arteritis in childhood: retrospective experience from a tertiary referral centre in the United Kingdom

Eleftheriou¹,

Varnier²,

Doležalová³

et al. 2015

Arthritis Res Ther

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“…Diagnosing TA relies on clinical presentation, characteristic structural arterial abnormalities, and evidence of inflammatory vasculopathy on imaging or histology. [8] Our patient presented with nonspecific symptoms of upper and lower limb pain and swelling of the body. Stenotic lesions predominate and tend to be bilateral.…”

Section: Discussionmentioning

confidence: 81%

Death due to severe occlusion of the subclavian artery; a case report

Lakmali¹,

Perera²,

Kumara³

et al. 2019

Medico-Legal J SL

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Takayasu arteritis is a rare, large vessel disease. It commonly affects aorta and its main branches. The characteristic features are absent or diminished pulse, vascular bruits, hypertension, chest pain, retinopathy, and dyspnoea. Some studies have shown that there is an association between hypothyroidism and Takayasu arteritis. We report a death of a 56-year-old female with suggestive symptoms of Takayasu Arteritis. She was diagnosed to have diabetes mellitus and ischaemic heart disease for 10 years. Lately, she was suffering from upper and lower limb pain. Three days before she died she developed severe limb pain and swelling of the body including upper and lower limbs. She was admitted to the tertiary care hospital and died on the following day. The electrocardiography showed subtle ischemic changes. Laboratory investigations were performed due to body swelling which confirmed hypothyroidism. At autopsy, there were severe occlusions of the left subclavian artery and severe stenosis of all coronary arteries. Histological examinations were performed since these findings are commonly seen in Takayasu arteritis. However, microscopy of Haematoxylin and Eosin stains revealed the occlusions in the left subclavian artery as atherosclerotic. Cause of death was given as atherosclerotic heart disease.

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“…Observation of granulomas and Langerhan's giant cells noted in tuberculosis also in TA patients suggests that possible tuberculosis infection may be involved in the etiology. A positive PPD test is observed frequently in patients with Takayasu arteritis (10).…”

Section: Takayasu Arteritismentioning

confidence: 98%

Diagnostic approach and current treatment options in childhood vasculitis

et al. 2015

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All inflammatory changes in the vessel wall are defined as vasculitis. Pediatric vasculitis may present with different clinical findings. Although Henoch-Schön-lein purpura which is the most common pediatric vasculitis generally recovers spontaneously, it should be monitorized closely because of the risk of renal failure. Although Kawasaki disease is easy to diagnose with its classical findings, the diagnosis may be delayed in case of incomplete Kawasaki disease. Kawasaki disease should be considered especially in infants in case of prolonged fever even if the criteria are not fully met and intravenous immunoglobulin treatment should be administered without delay in order to prevent development of coronary artery aneurism. Reaction at the site of administration of Bacillus Calmette-Guerin (BCG) vaccine may be observed as commonly as cervical lymphadenopathy in Kawasaki disease and may be used as a valuable finding in suspicious cases. Although anti-neutrophil cytoplasmic antibody-associated vasculitides are rare in children, renal involvement is more common and progression is more severe compared to adults. Hence, efficient and aggressive treatment is required. Takayasu's arteritis is observed commonly in young adult women and rarely in adolescent girls. Therefore, a careful physical examination and blood pressure measurement should be performed in addition to a detailed history in daily practice. In children with unexplained neurological findings, cerebral vasculitis should be considered in the absence of other systemic vasculitides and necessary radiological investigations should be performed in this regard. This review will provide an insight into the understanding of pediatric vasculitis, current diagnostic approaches and prognosis by the aid of new studies. (Turk Pediatri Ars 2015; 50: 194-205)

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Diagnosis and Assessment of Disease Activity in Takayasu Arteritis: A Childhood Case Illustrating the Challenge

Cited by 19 publications

References 30 publications

Takayasu arteritis in childhood: retrospective experience from a tertiary referral centre in the United Kingdom

Takayasu arteritis in childhood: retrospective experience from a tertiary referral centre in the United Kingdom

Death due to severe occlusion of the subclavian artery; a case report

Diagnostic approach and current treatment options in childhood vasculitis

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