A importância dos novos autoanticorpos específicos da dermatomiosite

  • Joana Calvão Interna de Dermatologia nos Hospitais da Universidade de Coimbra, Coimbra, Portugal
  • Alexandre Rafael Isidoro Azeiteiro Aluno da Faculdade de Medicina da Universidade de Coimbra, Coimbra, Portugal
  • Margarida Gonçalo 3Assistente Hospitalar Sénior de Dermatologia nos Hospitais da Universidade de Coimbra, e Professora Auxiliar convidada de Dermatologia da Faculdade de Medicina da Universidade de Coimbra, Coimbra, Portugal

Abstract

A dermatomiosite é uma miopatia inflamatória idiopática, rara, com manifestações cutâneas e sistémicas variadas. Mais de 70% dos doentes apresentam autoanticorpos específicos da doença, que se correlacionam com manifestações clínicas distintas. Além dos anticorpos anti-sintetase, nos últimos anos foram descobertos vários anticorpos associados à dermatomiosite e que parecem ter um papel importante na orientação diagnóstica e prognóstica da doença, incluindo os autoanticorpos contra melanoma differentiation antigen 5 (MDA5), transcriptional intermediary factor 1 (TIF1), nuclear matrix protein 2 (NXP2), small ubiquitin-like modifier activating enzyme (SAE) e os anti-Mi2. O autoanticorpo anti-MDA5 está associado a dermatomiosite amiopática e doença intersticial pulmonar rapidamente progressiva e potencialmente fatal, além de caraterísticas mucocutâneas distintas, como ulceração cutânea. Há uma forte correlação entre a positividade para os autoanticorpos anti-TIF-1γ e malignidade, pelo menos na dermatomiosite do adulto. Na dermatomiosite juvenil, estes autoanticorpos têm sido associados a doença cutânea mais extensa, mas não a malignidade. A positividade para anti-NXP2 está associada a calcinose cutânea e envolvimento muscular severos. Na dermatomiosite com autoanticorpos anti-SAE há frequentemente envolvimento cutâneo e disfagia severos, mas com boa resposta à terapêutica imunossupressora e bom prognóstico. Os anticorpos anti-Mi2 associam- -se igualmente a um bom prognóstico.

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Author Biography

Margarida Gonçalo, 3Assistente Hospitalar Sénior de Dermatologia nos Hospitais da Universidade de Coimbra, e Professora Auxiliar convidada de Dermatologia da Faculdade de Medicina da Universidade de Coimbra, Coimbra, Portugal

Assistente Hospitalar Sénior de Dermatologia nos Hospitais da Universidade de Coimbra, e Professora Auxiliar convidada de Dermatologia da Faculdade de Medicina da Universidade de Coimbra, Coimbra, Portugal

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Published
2019-03-26
How to Cite
Calvão, J., Azeiteiro, A. R. I., & Gonçalo, M. (2019). A importância dos novos autoanticorpos específicos da dermatomiosite. Journal of the Portuguese Society of Dermatology and Venereology, 77(1), 15-24. https://doi.org/10.29021/spdv.77.1.1021
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Continuous Medica Education