Autoantibody

Antigen Target

Associated Clinical Phenotype

Associated Cutaneous Features

Anti-Mi-2

Nuclear helicase

Reduced risk of interstitial lung disease, better response to therapy with fewer flares, overall good prognosis

Heliotrope rash, Gottron’s papules, V-and shawl-sign rashes, cuticular overgrowth

Anti-synthetase (anti-Jo-1, anti-PL-7, anti-PL-12, anti-EJ, anti-OJ, anti-KS, anti-Zo, others)

Aminoacyl tRNA synthetase

Fever, polyarthritis, interstitial lung disease, moderate to severe myositis; good response to corticosteroid therapy, but flare after tapering, less favorable prognosis

Mechanic’s hands, Raynaud’s

Anti-PM-Scl

PM-Scl complex, comprising 11-16 nucleolar polypeptides

Overlap with scleroderma, risk of pulmonary/renal disease

En coup de sabre, beak-like facies, severe Raynaud’s, and sclerodactyly

Anti-p155

155 kD protein  preliminarily identified as transcriptional intermediary factor 1 gamma

Common in adult and juvenile DM, malignancy associated DM , overlap myositis in DM , decreased risk of interstitial lung disease

Skin ulceration, erythroderma, edema, generalized lipodystrophy, V- and shawl-sign rashes, Gottron’s papules

Anti-CADM-140

140 kD protein of unknown specificity

Amyopathic  DM, increased risk of interstitial lung disease

“Classic cutaneous features of DM”—Heliotrope rash, Gottron’s papules

Anti-MJ

140 kD protein preliminarily identified as nuclear matrix protein NXP-2

Juvenile DM

Calcinosis