Background/Purpose:  Takayasu’s arteritis is a chronic inflammatory disease that affects the wall of large and medium-sized blood vessels. At the beginning of the disease, non-specific systemic symptoms predominate and in a more advanced phase of the disease, more characteristic signs and symptoms prevail. However atypical manifestations can occur, making the diagnosis difficult and leading to different misdiagnoses at the beginning of the disease. Therefore, our objective was to describe the main misdiagnoses and atypical manifestations in a multicenter study conducted on Brazilian children and adolescents.

Methods:  This Brazilian multicenter retrospective study included 71 children and adolescents with Takayasu’s arteritis diagnosed before their 19^th birthday and followed up in 10 pediatric rheumatology centers. Patients’ clinical, laboratorial and angiographic data were recorded.

Results:  Of the 71 patients, 51 (72%) were girls. The mean age at onset was 9.2 years and the mean time to diagnosis was 1.2 years. Eleven (15.5%) patients had diagnoses other than Takayasu’s arteritis at the beginning of the disease. The most common misdiagnosis was rheumatic fever, which was the initial diagnosis in 7 patients (2 of them had cardiac involvement). The other diagnoses before the correct diagnosis of Takayasu’s arteritis were spondiloarthropaty, juvenile idiopathic arthritis (JIA), arteriovenous malformation and JIA with polyarteritis nodosa. We also found 10 atypical manifestations of Takayasu’s arteritis, as follows: pyoderma gangrenosum (present in 2 patients), erythema nodosum, myositis, chorea, tendinitis, episcleritis, uveitis, hepatomegaly, splenomegaly and necrosis of extremities.

Conclusion:  Our study demonstrated that there are a variety of misdiagnoses that can be made at the beginning of the disease and also there are some atypical manifestations that can contribute to the difficulty in diagnosing Takayasu’s arteritis.