Table 1.
Clinical data of the cohorts and features from IBM patients muscle's biopsies
| Sample code (IBM) | Sex | Age | Time symptoms to diagnosis (months) | IBMFRS | Evolution | Rimmed vacuoles | Inflammation | MHC1 | Auto Ab | CD57 | Mitochondrial Changes | |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| IBM01 | F | 67 | 26 | 16 | Stability | + | + | NA | All − | Normal | No | |
| IBM02 | M | 51 | 48 | 16 | Progression | ++ | ++ | NA | All − | Increase | No | |
| IBM03 | M | 48 | 48 | 31 | Stability | + | + | NA | All − | NA | Yes | |
| IBM04 | F | 81 | 26 | 17 | Progression | +++ | + | NA | All − | Normal | Yes+ | |
| IBM05 | M | 63 | 36 | 27 | Progression | + | +++ | Universal | All − | NA | Yes + | |
| IBM06 | M | 77 | 48 | 28 | Progression | + | + | Neg | All − | Increase | Yes | |
| IBM07 | F | 61 | 36 | 23 | Progression | +++ | +++ | Universal | All − | NA | Yes | |
| IBM08 | F | 83 | 12 | 8 | Progression | + | +++ | NA | All − | T clonal | Yes + | |
| IBM09 | M | 62 | 84 | 30 | Progression | +++ | ++ | Universal | All − | Increase | Yes | |
| IBM10 | M | 78 | 12 | 28 | Progression | +++ | +++ | + Not universal | All − | NA | No | |
| IBM11 | F | 80 | 24 | 33 | Stability | +++ | + | Universal | Ro + | Normal | Yes + | |
| IBM12 | M | 71 | 36 | 30 | Stability | +++ | +++ | Universal | Ro52+ | Increase | Yes | |
| IBM13 | M | 75 | 72 | 32 | Stability | + | ++ | Universal | All − | Reduced B normal CD57 | Yes | |
| IBM14 | M | 40 | 3 | 33 | Stability | +++ | ++ | Universal | All − | NA | Yes+ | |
| Sample code (CTL) | CTL01 | CTL02 | CTL03 | CTL04 | CTL05 | CTL06 | CTL07 | CTL08 | CTL09 | CTL10 | CTL11 | CTL12 |
| Sex | M | F | F | F | F | F | M | F | F | M | M | M |
| Age | 69 | 66 | 61 | 41 | 48 | 47 | 35 | 45 | 82 | 86 | 57 | 52 |
| Summary | Fibroblasts (n) | Male/female ratio | Mean age (years) | Time to diagnose (months) | IBMFRS | |||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| IBM | 14 | 0.6 | 66.9 ± 3.6 | 37 | 25.1 ± 2.1/40 | |||||||
| CTL | 12 | 0.4 | 57.4 ± 4.6 | |||||||||
Note: Data presented as mean ± SEM.
Abbreviations: Sex: F, female; M, male; IBMFRS, Inclusion Body Myositis Functional Rating Scale (from 0 to 40 points). 20 Rimmed vacuoles: from 0 to +++; Inflammation: from 0 to +++; MHC1: Major Histocompatibility Complex: Universal; NA: not available; + not universal: splattered pattern; Auto Ab: Autoantibodies: Myositis specific and associated auto antibodies include: Anti‐Jo‐1, anti‐SRP, anti‐Mi2, anti‐TIF1gamma, anti‐MDA5, anti‐SAE, anti‐NXP2 and anti‐HMGCR (specific) and anti‐PM/Scl, anti‐Ro, anti‐La, and anti‐U1RNP (associated); Mitochondrial changes: Yes or No. Yes: RRF and/or COX neg. Yes + (strong alterations). No: no changes; Age and gender‐paired distribution between cohorts (P = NS).