Table 1: Muscle biopsy scores.

Domain Score
0 1 2 3
Inflammation
Inflammatory cell
infiltration
(endomysium: score×2)
None
(< 10 cells at 20× high power field)
1 cluster
(≥ 10 cells at 20× high power field)
2 clusters ≥ 3 clusters
Vascular
Vasculitis
none 1 vessel 2 vessels ≥ 3 vessels
Muscle
Atrophy
(perifascicular atrophy:
score ×2)
≤ 5% of total area 6–25% 26–50% > 50%
Degenerative fibers ≤ 5% of total area 6–25% 26–50% > 50%
Internal nuclei Absent
(≤ 3/100 fibers)
Present
(> 3/100 fibers)
Invaded non-necrotic
fibers
none 1 fascicle 2 fascicles ≥ 3 fascicles
Connective tissue
Endomysial fibrosis
none thin with angular fibers moderate thick with blunt fibers markedly thick with round fibers