Table 3 Rare autoantibodies in myositis and reported clinical associations.

Anti-ARS

1–30

Anti-synthetase syndrome

[69, 95]

Anti-Mi2

10

DM with typical skin lesions, mild disease

[69, 70, 95]

Anti-TIF1γ

10–15

Severe DM, malignancies

[70, 95]

Anti-NPX2

1–5

Severe DM, severe skin disease, malignancies

[69, 70, 95]

Anti-MDA5

15–20

CADM, ILD, severe skin manifestations, poor prognosis

[69, 95, 96]

Anti-SAE

1

Amyopathic DM

[95]

Anti-SRP

5

Necrotizing myopathy

[70]

Anti-HMGCR

6

Necrotizing myopathy, proximal muscle weakness, elevated CK levels, prior statin use

[70, 95, 97]