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Takayasu arteritis in childhood: misdiagnoses at disease onset and associated diseases

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Abstract

Juvenile-Takayasu arteritis (j-TA) is a difficult diagnosis and some patients develop uncommon manifestations and associated diseases that may contribute to the delayed diagnosis. Our aim was to identify the misdiagnoses, the associated diseases and the atypical manifestations observed in a j-TA Brazilian multicentre study. 71 children and adolescents who met the classification criteria for j-TA were included. The misdiagnoses, the associated diseases and the atypical manifestations were evaluated. 19 (26.8%) patients had misdiagnoses. The most common of them was aortic coarctation in six (8.4%) patients, followed by rheumatic fever in five (7.0%) and one patient presented with both former diagnoses. Limb pain (two patients), spondyloarthropathy, juvenile idiopathic arthritis (JIA), spinal arteriovenous malformation, polyarteritis nodosa (PAN) and fever of unknown origin (FUO) were other misdiagnoses. Patients who had misdiagnoses previously to j-TA diagnosis presented a trend to have a longer diagnosis delay. 11 (15.5%) patients had 14 TA-associated diseases, such as pulmonary tuberculosis (5 patients), rheumatic fever (2 patients), spondyloarthropathy, polyarticular JIA, Crohn’s disease, Prader–Willi disease, diabetes mellitus, Moyamoya and primary immunodeficiency. 7 (9.9%) patients presented 10 atypical manifestations, such as pyoderma gangrenosum, erythema nodosum, myositis, chorea, enthesitis, episcleritis, uveitis, hepatomegaly, splenomegaly and necrosis of extremities. Our study emphasizes the main misdiagnoses, associated diseases and atypical manifestations that occur in patients with j-TA and warns of the features that may alert paediatricians to this diagnosis, such as constitutional symptoms and elevated inflammatory markers.

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Funding

This study has received no funding.

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Authors and Affiliations

Authors

Contributions

GC conceived the sudy, collected the data, analyzed the data and drafted the manuscript. CAS, SBS, VPLF, SKO, FS, BERGB, AC, TR and MB collected their own data and reviewed the manuscript. MTT conceived the study, coordinated the study and reviewed the manuscript. All authors agreed with the final version.

Corresponding author

Correspondence to Gleice Clemente.

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Conflict of interest

Gleice Clemente, Clovis A Silva, Silvana B Sacchetti, Virginia P L Ferriani declares, Sheila K Oliveira, Flavio Sztajnbok, Blanca E R G Bica, André Cavalcanti, Teresa Robazzi, Marcia Bandeira and Maria Teresa Terreri declare that they have no conflict of interest.

Ethical approval

This study was approved by the Research Ethics Committees of all participating centres.

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Clemente, G., Silva, C.A., Sacchetti, S.B. et al. Takayasu arteritis in childhood: misdiagnoses at disease onset and associated diseases. Rheumatol Int 38, 1089–1094 (2018). https://doi.org/10.1007/s00296-018-4030-4

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  • DOI: https://doi.org/10.1007/s00296-018-4030-4

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