Takayasu Arteritis: A 4-year-old girl presenting with Heart Failure - Case Report and Literature Review (original) (raw)
Takayasu arteritis in children
Pediatric Rheumatology, 2008
Takayasu arteritis (TA) is a large vessel vasculitis that usually affects young female patients during the second and third decades of life, but has been reported in children as young as 24 months of age. Aim of this report was to describe four children (two girls) with TA, as well as summarizing main published studies. The mean age at presentation of our cases was 11 years (range 8-15). Three patients were Caucasians and one Asian. Arterial hypertension was the commonest mode of presentation followed by systemic symptoms. Other related symptoms were due to ischemia and consisted of abdomen, chest, and limb pain. An abdominal bruit was noted in only one patient. Inflammation markers were always abnormal. Angiography was performed in all cases; left subclavian artery and common carotid artery were more frequently involved. Renal artery stenosis was observed in two patients. One boy was diagnosed as having an associated immune deficiency (Wiskott-Aldrich syndrome). Treatment modalities included prednisone (n = 4), methotrexate (n = 3), and mycophenolate mofetil (MMF) (n = 1). Surgery was required in two patients. Follow-up ranged from 3 to 10 years since diagnosis. In three cases antihypertensive drugs and methotrexate were stopped, and prednisone was reduced to 7.5 mg/day.
Atypical childhood Takayasu arteritis -A case report
Indian Journal of Pharmacy and Pharmacology, 2023
Childhood Takayasu arteritis is one of the most common vasculitis in paediatric age group. It predominantly involves the greater vessels such as aorta and its branches, leading to granulomatous inflammation. The process of inflammation gives rise to the symptoms based on thrombotic or aneurysmal phenomena. This is a case report of a 11-year-old girl diagnosed in an urban tertiary care centre in India with childhood Takayasu arteritis. The said child had first presented with non-specific symptoms such as leg ache, back ache and fever. Over the course of illness, the child developed hypertension and had also suffered from PRES (posterior reversible encephalopathy syndrome). Clinical examination was consistent with Takayasu arteritis, however with predominant medium-sized vessel involvement on imaging. Since this is a relatively atypical presentation, it was mandatory to rule out other causes of medium-vessel arteritis, especially DADA2 (deficiency of ADA2). Keywords: Takayasu’s arteritis, DADA2, Vasculitis
Takayasu arteritis in childhood: retrospective experience from a tertiary referral centre in the UK
Arthritis Research & Therapy, 2015
Introduction: Takayasu 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. Methods: We 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. Results: A 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). Conclusion: TA 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.
A Rare Case of Childhood Arteritis
2015
Takayasu’s arteritis (TA) is a chronic, progressive, autoimmune, idiopathic, large-vessel vasculitis that usually affects young adult females. The disease has been reported to occur in all races and ethnicities. The diffuse nature of this disease can affect multiple-organ systems to varying degrees. Herein, we report the case of a nine year old child who presented with diarrhea, vomiting, loss of weight and weakness. Due to her initial presentation and lab investigations, she was started on antituberculous therapy. During further investigations she was diagnosed to be suffering from Takayasu’s arteritis. After one year of ongoing treatment and follow-up, she displayed marked resolution in disease process affecting abdominal aorta and its main terminal branches. We discuss a possible relationship between Takayasu’s arteritis and both latent and active tuberculosis which was first pointed out in 1948 by Shimizu and Sano.
A five years old girl child with takayasu arteritis
Takayasu arteritis is a systemic vasulitis of large vessels that mainly involves the aorta and its branches. It normally presents in third decade of life and has rarely been reported in children under 10 years of age. We report here a case of Takayasu arteritis in a 5 years old girl who presented with headache, generalized body swelling, severe hypertension, proteinuria and minimal functioning kidneys. Conventional angiography demonstrated narrowing of descending aorta, right subclavian artery and right common iliac artery. She responded steroids, diuretics, antiplatelets and digoxin and discharged home on maintenance therapy.
Aggressive Progression of Takayasu’s Arteritis in Infancy: A Case Report
Acta Clinica Croatica, 2019
-Takayasu's arteritis (TA) affects the aorta and its branches. immunosuppressants are the usual course of therapy, while surgery has been used in acute cases. There is only scant information on TA in infancy, and the nonspecific symptoms in the initial stage of the disease make the diagnosis difficult and delayed, thus increasing the mortality rate. We describe a case of aggressive progression of TA in an infant. This child was the youngest to be affected with the disease as reported in the literature. A 3.5-month-old boy presented with cyanosis of both legs, tachycardia and antithrombin iii deficiency. Computed tomography angiography (CTA) revealed thrombosis of distal aorta and both iliac arteries. Thrombectomy was performed at the level of both common femoral arteries. in addition, thigh amputation of the left leg had to be performed. TA was diagnosed postmortem with thrombosis of the distal aorta, its branches and upper mesenteric artery which was not occluded on previous CTA, glomerulonephritis and pulmonary parenchymal granulomatous infiltrations.
University Heart Journal, 1970
Takayasu's arteritis or pulseless disease is a chornic large vessel vasculities the cause of which is largely unknown. Takayasu's arterities is seldom reported, because it is a rare disease affecting about only 3 (three) people of every million indivisuals throughout the world, most commonly among Asian female of childbearing age. It rarely causes happertension in children. We present an 11 years old girl without typical symptomatic manifestation and with a unique presentation of headache & swelling of the body for one year. She also complainted of examinational dyspnoea for same duration and abdominal pain for six months. Her clinical examination revealed very feeble left upper limb pulse with no radio-femoral delay, different blood pressure of both arms and limbs, systolic bruits audible in left 2nd intercostal space and grade-1 apical pansystolic murmur. Her coronary angiogram report showed diffuse narrowing of a long segment of abdominal aorta, severe stenosis of proxima...
Is Takayasu's arteritis more severe in children?
Clinical and Experimental Rheumatology
Objective. Takayasu's arteritis (TAK) is a chronic vasculitis, affecting predominantly the aorta and/or its major branches. The aim of this study was to compare the differences between childhood and adult onset TAK. Methods. We retrospectively evaluated 179 TAK patients followed between August 2005 and July 2019. Demographic characteristics, laboratory features, disease activity, echocardiographic data at diagnosis and treatment regimens in the disease course were compared between the paediatric and adult onset patients. Results. Twenty-five paediatric-onset (<18 years of age at diagnosis) and 154 adult-onset patients (≥18 years of age at diagnosis) were enrolled. The mean age at diagnosis for children and adults were 13.6±4 and 35.6±13, respectively. Paediatric onset TAK patients had more intense inflammation at the time of diagnosis reflected in their clinical findings. Acute phase reactants were high in all paediatric patients and significantly higher in patients with paediatric-onset TAK (p=0.006 and p=0.005, respectively). Abdominal predominant disease was more common in the paediatric group, in contrast, focal disease and aortic arch predominant disease were more common in the adult group. Ascending aortic dilatation, left ventricular hypertrophy and moderate-severe aortic insufficiency were more frequent in echocardiography findings of paediatric onset TAK patients. In comorbidities, hypertension was more common in paediatric TAK patients during followup, whereas cerebrovascular disease was more common in adult patients. Conclusion. Our paediatric onset TAK patients presented with a more severe inflammation and more widespread vascular involvement. Multicentre studies from different geographic areas are needed to verify our observation and understand the underlying causes. Methods Study protocol and patients We retrospectively evaluated the medical records of TAK patients followed at
Girl with Takayasu Arteritis -Progressive Course of the Disease and Multiple Surgical Interventions
Acta medica Croatica : Časopis Akademije medicinskih znanosti Hrvatske, 2020
Takayasu arteritis is a rare large-vessel vasculitis but it can be associated with high mortality rates in childhood. Granulomatous vasculitis usually affects the aorta and/or main branches but also coronary and pulmonary arteries. The course of the disease is unpredictable and management is based on controlling infl ammation and preventing end-organ damage. In this case we describe a patient with progression of vasculitis but so far successful prevention of possible ischemic consequences using immunosupressive and biologic therapy and multiple surgical interventions over the course of the disease. A 14-year-old girl presented with precordial pain and numbness of the left arm. Physical examination revealed the absence of the radial pulse in the left arm. Computed tomography angiography showed subtotal occlusion of the left main coronary artery, subtotal occlusionof the left common carotid artery, subtotal occlusion of the left subclavian artery and stenosis of thoracic aorta below isthmus of aortae. Despite aggresive conservative therapy and cardiosurgical treatment the course of the disease was complicated with restenoses which were resolved with subsequent revascularization procedures. Here we present an adolescent girl with progressive vasculitis and with multiple surgical interventions. Carefully monitoring of the patient and good collaboration between pediatric cardiologist and rheumatologist with radiologists and cardiac surgeons improved life-quality of the patient which now studies at the University and has good physical and mental status.
Childhood-onset Takayasu arteritis: an update
International Journal of Rheumatic Diseases, 2015
Childhood-onset Takayasu arteritis (c-TA) is a distinct subset affecting a wide age group, ranging from young infants to adolescents and it differs from adult TA in many aspects. There is scarcity of data on c-TA worldwide. The disease is classified using the European League Against Rheumatism/Pediatric Rheumatology International Trials Organization/Pediatric Rheumatology European Society criteria. The non-specific nature of presenting complaints and lack of appropriate biomarkers delay the early diagnosis of this illness and many children present with complications, which become irreversible once they set in. One of the largest cohorts of 40 children with c-TA from our center reports hypertension as the commonest presenting feature. Systemic symptoms like headache, fever and weight loss are also described. Assessment of disease in c-TA is done by correlating clinical features with raised inflammatory markers. Advanced imaging plays an important role in diagnosis. In c-TA, the role of magnetic resonance angiography is advocated, taking into consideration the enormous amount of radiation exposure with other modalities. Complications of c-TA include cardiovascular, pulmonary, neurological and those arising secondary to long-term steroid and immunosuppression therapy.