Autoimmune response to transthyretin in juvenile idiopathic arthritis.

Juvenile idiopathic arthritis (JIA) is the most common pediatric rheumatological condition. Although it has been proposed that JIA has an autoimmune component, the autoantigens are still unknown. Using biochemical and proteomic approaches, we identified the molecular chaperone transthyretin (TTR) as an antigenic target for B and T cell immune responses. TTR was eluted from IgG complexes and affinity purified from 3 JIA patients, and a statistically significant increase in TTR autoantibodies was observed in a group of 43 JIA patients. Three cryptic, HLA-DR1-restricted TTR peptides, which induced CD4+ T cell expansion and IFN-γ and TNF-α production in 3 out of 17 analyzed patients, were also identified. Misfolding, aggregation and oxidation of TTR, as observed in the synovial fluid of all JIA patients, enhanced its immunogenicity in HLA-DR1 transgenic mice. Our data point to TTR as an autoantigen potentially involved in the pathogenesis of JIA and to oxidation and aggregation as a mechanism facilitating TTR autoimmunity.

Non-syndromic hypohyperdontia of the permanent dentition with involvement of the mandibular anterior region: a rare occurrence / Hipo-hiperdontia não sindrômica da dentição permanente com envolvimento da região anterior mandibular: uma rara ocorrência

Objective: To present an uncommon case of numeric abnormalities in a non-syndromic patient.Discussion: Dental numeric abnormalities in development of the permanent dentition are quite common;however, combined occurrence of hypodontia and hyperdontia is a rare phenomenon, especiallyin a non-syndromic situation. This paper describes a 28-year-old non-syndromic man with missing mandibularlateral incisors and an erupted mandibular mesiodens. This is only the third case, to the best ofour knowledge, with the two anomalies manifesting in the anterior region of the mandible.

Objetivo: Apresentar um caso incomum de anomalias numéricas num caso de paciente não sindrômico. Discussão:Anomalias dentárias numéricas no desenvolvimento da dentição permanente são comuns; entretanto, a ocorrênciacombinada de hipo e hiperdontia é um fenômeno raro, especialmente em situações não sindrômicas. Este artigo descreve o caso de um paciente masculino não sindrômico apresentando ausência de incisivos laterais mandibularese um mesiodens mandibular erupcionado. Esse é o único caso, pelo melhor de nosso conhecimento, com as duasanomalias manifestando-se simultaneamente na região anterior da mandíbula.