Distinctive cutaneous and systemic features associated with specific anti-myositis antibodies in adults with dermatomyositis: a prospective multicentric study of 117 patients

Abstract

Background

Identification of myositis-specific autoantibodies (MSA) for dermatomyositis (DM) could allow the characterization of an antibody-associated clinical phenotype.

Objective

We sought to define the clinical phenotype of DM and the risk of cancer, interstitial lung disease (ILD) and calcinosis based on MSA.

Methods

A 3.5-year multicenter prospective study of adult DM patients was conducted to determine the clinical phenotype associated with MSA and the presence of cancer, ILD and calcinosis.

Results

MSA were detected in 47.1% of 117 included patients. Patients with anti-melanoma differentiation-associated protein 5 antibodies (13.7%) had significantly more palmar violaceous macules/papules (odds ratio (OR) 9.9), mechanic's hands (OR 8), cutaneous necrosis (OR 3.2), and articular involvement (OR 15.2), and a higher risk of ILD (OR 25.3). Patients with anti-transcriptional intermediary factor-1 antibodies (11.1%), anti-nuclear matrix protein-2 antibodies (6.8%) and anti-aminoacyl-transfer RNA synthetase (5.1%) had, respectively, significantly more poikiloderma (OR 5.9), calcinosis (OR 9.8), and articular involvement (OR 15.2). Cutaneous necrosis was the only clinical manifestation significantly associated with cancer (OR 3.1).

Conclusion

Recognition of the adult DM phenotype associated with MSA would allow more accurate appraisal of the risk of cancer, ILD and calcinosis.

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