Childhood-onset Takayasu arteritis(c-Tak) is a rare chronic granulomatous disease of the large vessel seen in children. It involves the aorta, its branches, and the pulmonary arteries [1]. The large vessels undergo stenosis, occlusion, or dilatation following inflammation. The exact etiology and pathogenesis remain unknown. Its spectrum of presentation varies and is mainly due to vascular occlusion leading to end-organ ischemia. According to revised criteria in 2010[2], in children, angiographic abnormality, with aneurysm or dilatation, narrowing, occlusion, or thickening of the aorta or its main branches, is a mandatory criterion for the diagnosis. In addition to this mandatory criteria, at least one of the following should be fulfilled: pulse deficit or claudication, blood pressure discrepancy > 10 mm Hg in any limb, hypertension with systolic or diastolic > 95th centile for height, and erythrocyte sedimentation rate (ESR) > 20 mm/h or elevated C-reactive protein (CRP). Here we are reporting case series of Takayasu arteritis in children with varied presentations.

A 15-year-old, developmentally normal, well immunized female child was admitted in April 2019 with complaints of palpitation and fatigue on mild activity for 7-8months. She developed palpitation and weakness of all four limbs on exertion. Each episode lasted for 3-5mins which improved on taking rest. The symptoms were non-progressive and equal in all four limbs. There was no history of chest pain, claudication, headache, vomiting, fever, excessive sweating. She is a known case of hypertension and on antihypertensives carvedilol, chlorothiazide, and prazosin for eight months. She had irregular menses with a 1-2 months gap with a normal flow of 2-3 days without pain. Her appetite was normal. On General examination, the child was conscious, alert with a normal gait. Her pulse rate was 112/min, well felt in the upper limb, not palpable in the lower limb. Her BP in right upper limb-160/120mm Hg, left upper limb-160/120mmHg, right lower limb110/88mmHg, Left Lower limb-100/66mm Hg. Her respiratory rate was 18/min, and the temperature was 98^◦F. There was mild pallor, no icterus, no cyanosis, clubbing or lymphadenopathy, no edema, JVP was not raised. Her height was152.4 cm, and her weight was 33kg which comes under moderate malnutrition.

On examination, the child was afebrile, pulse was 60 /min, and upper limb and lower limb BP differences were not noted. Peripheral pulses were well felt, and the rest of the examination was normal. The patient was initially on MMF 500. He was worked up for disease activity by CRP, ESR, which came to be normal. ECG and Echo were normal. There is no history of breathlessness. CT angiography of coronary and thoracic was done, which was reported to be normal.

The child was started on methotrexate @10mg/m2/week, and MMF was tapered. Oral Prednisolone was given for five days and stopped. As a family stressor was present and clinically, we could not elicit any cause of new-onset pain, hence Psychiatry opinion was done and was diagnosed to be Acute stress disorder VS adjustment disorder and started on Escitalopram and Clonazepam. His investigation showed normal CBC, RFT, LFT and normal ESR, CRP with only insufficiency of Vit D.

7 yr female child born out of non-consanguineous marriage, developmentally normal, got admitted on Oct 1, 2019, with 1 episode of seizure three days back, associated with loss of consciousness and weakness of right side of body and deviation of angle of mouth. The child had a history of right-sided weakness one year back and recovered gradually. She had taken some ayurvedic medication.

On examination, the child was conscious, oriented, pulse 80/min, not palpable in upper limb but palpable in the lower limb.BPwas 88/72mm Hg in the upper limb and 138/90 mm Hg in the lower limb. Her respiratory rate was 20/m, and she was afebrile.

There was mild pallor, no icterus, no cyanosis, no clubbing, no lymphadenopathy. Child weight was 17kg, height was 114cm s/o underweight. Right-sided hemiparesis was present with power 3/5, exaggerated reflex, and extensor plantar on the right side. Left sidetone, power, reflex were normal with plantar flexor with Rt UMN type facial palsy. A provisional diagnosis of Takayasu arteritis with stroke was made. She was started on in. Levetiracetam, aspirin, and given NG feeding, fundus showed no hypertensive changes in the retina. Amlodipine was started, and BP was controlled to 90^th centile.

Her CBC showed leucocytosis with neutrophilic preponderance. Her ESR was 80, CRP was 60. Blood C/S was sterile. Hence she was started on antitubercular therapy with a plan of the initiation phase of 2 months of HRZE followed by six months of continuation phase with HRE.

Childhood Takayasu disease is 3^rd most common vasculitis among children after Henoch schooling Purpura and Kawasaki disease. To diagnose c-TAK, currently, EULAR/PRINTO/PRES childhood TAK (c-TAK) classification criteria (Table 2) are being used, which has sensitivity and specificity around 100% and 99.9%, respectively.[2].

It has a 2:1 female: male preponderance according to the Indian and South African series. [8,9]

In our case series, also females (3/4)outnumber male children.

CT: Computer Tomography; CRP: C-reactive protein; EULAR: European League Against Rheumatism; MRI: Magnetic Resonance Imaging; PRES: Pediatric Rheumatology European Society; PRINTO: Pediatric Rheumatology International Trials Organization

We observed HTN in 3/4 cases, stroke in 2/4 of patients. As described by various literature, hypertension has been the most common symptom in adults and children, and stroke has been described in 17% cases in c-TA and 20-24 % in patients with TA. [10]. Due to peripheral vascular obstruction, often peripheral pulses are not well felt, so it is called 'pulseless disease'.We noticed pulse deficits in ¾ cases. One of the cases was diagnosed to have tuberculosis and started on ATT. There is an association between tuberculosis and Takayasu arteritis in developing countries where tuberculosis (TB) prevalence is more common. There is a description of active tuberculosis up to 20% in adult TA patients. [11]. A strongly positive Mantoux test has been observed in 20% of all TA patients in one study from India. [12]. Whether this association represents a causal relationship or a mere coincidence is still unclear.

Imaging findings: Vascular imaging is essential for the establishment of diagnosis and management of c-TA cases. Table 3 shows the angiographic classification of Takayasu Arteritis. Imaging modalities can be Conventional angiography, Magnetic Resonance Angiography(MRA), Computed Tomography Angiography(CTA), Doppler Ultrasound, and fluorodeoxyglucose positron emission tomography (PET).

Early evaluation by angiography is important as it guides surgical intervention also. The active disease process has to be managed with corticosteroids and immunosuppressive agents.