Table 1

Score points for the European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies to be used when no better explanation for the symptoms or signs exists1

VariableScore points
No biopsyBiopsy
Age of onset of first related symptoms
Muscle weakness
 Objective symmetric weakness, usually progressive, of proximal upper extremities0.70.7
 Objective symmetric weakness, usually progressive, of proximal lower extremities0.80.5
 Neck flexors are relatively weaker than neck extensors1.91.6
 In the legs, proximal muscles are relatively weaker than distal muscles0.91.2
Skin manifestations
 Heliotrope rash3.13.2
 Gottron’s papules2.12.7
 Gottron’s sign3.33.7
Other clinical manifestations
 Dysphagia or esophageal dysmotility0.70.6
Laboratory measurements
 Anti-Jo-1 (anti-histidyl-tRNA synthetase) autoantibody positivity3.93.8
 Elevated serum levels of creatine kinase (CK)* or lactate dehydrogenase (LDH)* or aspartate aminotransferase (ASAT/AST/SGOT)* or alanine aminotransferase (ALAT/ALT/SGPT)*1.31.4
Muscle biopsy features
 Endomysial infiltration of mononuclear cells surrounding, but not invading, myofibres1.7
 Perimysial and/or perivascular infiltration of mononuclear cells1.2
 Perifascicular atrophy1.9
 Rimmed vacuoles3.1
  • *Serum levels above upper limit of normal.