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Inês Pizarro Madureira Salgado Da Costa1, José Diogo Martins2, Margarida Paula Ramos1

1 - Unidade de Reumatologia, Área da Mulher, Criança e Adolescente, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisboa
2 - Serviço de Cardiologia Pediátrica, Hospital de Santa Marta, Centro Hospitalar Universitário de Lisboa Central, Lisboa

- 25th European Paediatric Rheumatology Congress, Lisboa, 5 a 8 de setembro de 2018, poster

Introduction: Takayasu arteritis is an idiopathic large-vessel vasculitis that primarily affects the aorta and its major branches. Chronic granulomatous inflammation leads to stenosis and occasionally aneurysms of the involved portions of the arteries, producing a wide variety of symptoms. High-dose corticosteroids are the mainstay of therapy but adjunct immunosuppression is frequently required. TNF-α blocking agents have shown to be effective in refractory cases.
Objectives: Characterization of patient demographics, clinical features, disease extent, treatment and outcomes in children with Takayasu arteritis.
Methods: Retrospective data analysis of a case series of children fulfilling the EULAR/PRINTO/PRES criteria for childhood Takayasu arteritis in a tertiary hospital setting during a 10-year period.
Results: A total of 4 children (3 female), with a median age at diagnosis of 7 years (5 -10 years) were identified during this period. The most common clinical features at presentation were systemic symptoms and cardiovascular involvement (4/4). Weak peripheral pulses, discrepant systolic blood pressure between limbs and arterial bruits were present in all cases. Arterial hypertension was detected in half of the patients. Neurological manifestations were also very common (3/4), with seizures at the initial presentation in 2 patients and ischemic stroke in 1 case. A minority of patients complained of gastrointestinal (1/4) and respiratory (1/4) symptoms. Acute phase reactants (ESR and CRP) were elevated in all patients. Thrombocytosis (3/4) and chronic disease anaemia (2/4) were also commonly found. The distribution of the involved arteries according to the angiographic classification of Takayasu arteritis showed a pattern type V in 3 cases, one of which was VP+. The remaining case displayed a pattern IIa. First-line treatment in all cases included high-dose corticosteroids and methotrexate. Cyclophosphamide was added in 2 cases. Due to a severe and refractory disease course, TNF-α blocking agents were administered in all patients (Infliximab, 2 cases; Adalimumab, 2 cases). Endovascular procedures were required in 2 patients with irreversible stenosis: renal artery percutaneous transluminal angioplasty (2 cases) and pulmonary artery stenting (1 case). There were no relevant infections or mortality during the follow-up period. Repeated magnetic resonance angiography to assess disease activity showed improvement in vascular lesions in 3 patients, with remission in 1 case. None of the patients presented disease progression with involvement of new vessels.
Conclusion: The diagnosis of childhood Takayasu arteritis remains challenging, mainly due to the insidious clinical course and the lack of specificity of the presenting symptoms. The abnormal peripheral pulses in the setting of elevated inflammatory markers should raise suspicion for this condition. Previous studies found that constitutional features like low-grade fever, weight loss and arthralgia are more frequently found in childhood Takayasu arteritis compared with adult patients. In our series these symptoms were present in all patients. We report a higher frequency of neurological involvement compared with other paediatric reports, perhaps reflecting the severity of the angiographic pattern and the delay in diagnosis. None of our patients achieved stable remission with high-dose corticosteroids and methotrexate only.  Anti-TNFα showed to be beneficial in refractory patients with clinical improvement in the majority of the cases, as previously reported in other studies.

Palavras Chave: Biologic Therapy, Children, Takayasu Arteritis