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- Title
A Importância dos Novos Autoanticorpos Específicos da Dermatomiosite.
- Authors
Calvão, Joana; Isidoro Azeiteiro, Alexandre Rafael; Gonçalo, Margarida
- Abstract
Dermatomyositis is a rare idiopathic inflammatory myopathy with diverse cutaneous and systemic manifestations. Over 70% of patients have disease-specific autoantibodies that correlate with distinct clinical manifestations. In recent years, several antibodies have been discovered and appear to play an important role in the diagnostic and prognostic orientation of the disease, including autoantibodies against melanoma differentiation antigen 5 (MDA5), transcriptional intermediate factor 1 (TIF1), nuclear matrix protein 2 (NXP2), small ubiquitin-like modifier activating enzyme (SAE) and anti-Mi2. The anti-MDA5 autoantibody is associated with amyopathic dermatomyositis and rapidly progressive and fatal pulmonary interstitial disease, as well as distinct mucocutaneous features such as cutaneous ulceration. There is a strong correlation between positivity for anti-TIF-1γ autoantibodies and malignancy, at least in adult dermatomyositis. In juvenile dermatomyositis, these autoantibodies have been associated with more extensive cutaneous disease, but not malignancy. Anti-NXP2 positivity is associated with severe cutaneous calcinosis and muscle involvement. In dermatomyositis with anti-SAE autoantibodies there is often severe cutaneous involvement and dysphagia, but with a good response to immunosuppressive therapy and good prognosis. Anti-Mi2 antibodies are also associated with a favourable prognosis.
- Subjects
NUCLEAR matrix; MYOSITIS; SKIN diseases; DERMATOMYOSITIS; AUTOANTIBODIES; CALCINOSIS
- Publication
Revista da Sociedade Portuguesa de Dermatologia e Venereologia, 2019, Vol 77, Issue 1, p15
- ISSN
2182-2395
- Publication type
Article
- DOI
10.29021/spdv.77.1.1021