Síndrome de Erasmus Associada à Policondrite Recidivante: Relato de Caso / Erasmus’ Syndrome in Association with Relapsing Polychondritis: a Case Report
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Abstract
Introdução: A silicoesclerodermia foi descrita pela primeira vez por Erasmus, e é consequência de uma resposta inflamatória desencadeada pela sílica, envolvendo a liberação de mediadores que causam alteração na imunidade celular e humoral, levando a formação de auto-anticorpos e imunocomplexos circulantes. A policondrite recidivante é uma doença sistêmica e auto-imune, caracterizada por processo inflamatório recorrente que causa destruição de cartilagens de todo o corpo. Casuística: Trata-se de um caso de um paciente do sexo masculino, de 57 anos, que desenvolveu policondrite recidivante e durante sua investigação foi diagnosticada a síndrome de Erasmus. Conclusão: este trabalho relatou um caso em que foi observada a associação de duas patologias raras: a síndrome de Erasmus e a policondrite recidivante, doenças que possuem manifestações potencialmente fatais, sendo muito importante o seu reconhecimento, visando o diagnóstico precoce e tratamento. Não foi encontrada na literatura a associação entre elas.
Palavras Chave: policondrite recidivante, silicose, escleroderma sistêmico
Introduction: Silicoscleroderma was described for the first time by Erasmus, and it is a consequence of an inflammatory response caused by silica, involving liberation of mediators which cause changes in cellular and humoral immunity, conducting to the formation of auto-antibodies and circulating immune complexes. Relapsing polychondritis is a systemic and auto-immune disease, characterized by recurrent inflammatory process which causes destruction of all body cartilage. Reporting: This is a case of a male patient of 57 years who developed relapsing polychondritis and during its investigation the Erasmus' syndrome was diagnosed. Conclusion: This work related a case in which the association of two rare diseases was observed: Erasmus' syndrome and relapsing polychondritis, diseases that have potentially fatal complications, it is very important their recognition, for the early detection and treatment. Was not found in the literature regarding the association between them.
Keywords: relapsing polychondritis, silicosis, systemic scleroderma
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