Table 3.

Main muscle biopsy features of sIBM patients carrying variants in SQSTM1 and VCP genes

Muscle biopsy features	Case 1	Case 2	Case 3	Case 4	Case 5	Case 6	Case 7
Endomysial exudate	+	+	+	+	+	+	+
MHC-I upregulation	+	+	+	+	+	+	+
Partial invasion	+	+	+	−	+	−	−
Rimmed vacuoles	+	+	+	+	+	−	−
p62 (sarcoplasmic and intranuclear inclusions)	+	+	+	N/A	N/A	N/A	N/A
TDP-43	N/A	+	N/A	N/A	N/A	N/A	N/A
15–18 (or 16–21) nm filaments	N/A	N/A	N/A	−	N/A	N/A	N/A
COX-deficient fibers	+	+	−	+	−	+	+

Key: COX, cytochrome c oxidase; MHC, major histocompatibility complex; N/A, not available; sIBM, sporadic inclusion body myositis; SQSTM1, sequestosome 1; TDP-43, TAR DNA-binding protein-43; VCP, valosin-containing protein.