Abstract
BackgroundTo analyze the frequency and clinical relevance of anti-Mi-2 autoantibody in a representative sample of patients with dermatomyositis.MethodsThis longitudinal inception cohort study, from 2001 to 2017, included 87 definite adult dermatomyositis. Anti-Mi-2 analysis was performed using a commercial kit.ResultsSeventeen patients (19.5%) had anti-Mi-2 and 70 (80.5%) did not have this autoantibody. The following parameters were equally distributed between the patients with versus without anti-Mi-2: mean age at the disease diagnosis onset, median follow-up time, constitutional symptoms (baseline), cutaneous cumulative lesions, dysphagia, joint and pulmonary involvement. There was also no difference between the groups in relation to follow-up time, disease relapsing, treatment, disease status, deaths and occurrence of neoplasia. In contrast, patients with anti-Mi2 antibodies had higher frequency of elevated serum levels of muscle enzymes at disease onset (median: creatine phosphokinase 6240 [3800–9148] U/L and aldolase 60.0 [35.0–138.0] U/L), lower frequency of pulmonary involvement at disease onset (5.9%), less current glucocorticoid dose (median: 0 [0–10] mg/day), and higher frequency of disease remission during follow-up (58.8%) in comparison with patients without anti-Mi-2 autoantibody (484 [115–4880] and 12.1 [6.3–70.0] U/L, 40.0%, 0 [0–10] mg/day, 27.1%, respectively).ConclusionThe anti-Mi-2 autoantibody was found in one fifth of patients with dermatomyositis. This autoantibody was associated with a lower occurrence of pulmonary involvement, a higher frequency of disease in remission, and elevated levels of muscle enzymes. There was also no correlation regarding the frequency of disease relapsing or neoplasia development.
Highlights
Dermatomyositis (DM) is a systemic autoimmune myopathy that primarily affects the skin and skeletal striated muscle [1,2,3,4,5]
Several autoantibodies have been described in DM, which can be classified into myositis-specific or associated [2, 7,8,9,10,11,12,13,14,15,16,17,18]
Among the myositis-specific autoantibodies, there are controversies in the literature, anti-Mi-2 autoantibody has been correlated with the presence of cutaneous lesions [2, 7, 9,10,11,12,13,14], absence of pulmonary fibrosis [7, 9, 10, 13, 15], low risk of malignancy [7, 9, 13,14,15] and good disease outcome with glucocorticoid treatment [9, 13, 15]
Summary
Dermatomyositis (DM) is a systemic autoimmune myopathy that primarily affects the skin and skeletal striated muscle [1,2,3,4,5]. Among the myositis-specific autoantibodies, there are controversies in the literature, anti-Mi-2 autoantibody has been correlated with the presence of cutaneous lesions [2, 7, 9,10,11,12,13,14], absence of pulmonary fibrosis [7, 9, 10, 13, 15], low risk of malignancy [7, 9, 13,14,15] and good disease outcome with glucocorticoid treatment [9, 13, 15] These studies were based on patients with DM and on other inflammatory myopathies, which made it difficult to make precise conclusions about the reactivity of anti-Mi-2 autoantibody in patients with DM. To analyze the frequency and clinical relevance of anti-Mi-2 autoantibody in a representative sample of patients with dermatomyositis
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