Table 1.
Patient or control | Diagnosis | Age (years) | Sex | Biopsied muscle | No. of isolated myofibers to reach >100,000 μm2⁎ |
---|---|---|---|---|---|
Patient no. | |||||
IBM-1 | sIBM | 75 | Male | Biceps brachii | 43/37 |
IBM-2 | sIBM | 70 | Male | Vastus lateralis | 42/35 |
IBM-3 | sIBM | 79 | Female | Biceps brachii | 42/38 |
IBM-4 | sIBM | 69 | Male | Triceps brachii | 50/41 |
IBM-5 | sIBM | 72 | Male | Triceps brachii | 50/47 |
Control no. | |||||
C-1 | Nonspecific myalgia, depression | 57 | Male | Biceps brachii | 55 |
C-2 | Nonspecific myalgia | 61 | Male | Biceps brachii | 60 |
C-3 | Nonspecific myalgia | 52 | Male | Rectus femoris | 57 |
Five patients with sIBM and three controls were included in the study. Their clinical and histopathological characteristics are listed. The congophilic deposits were visualized in Congo red–stained sections viewed under rhodamine optics. All biopsy specimens of patients with sIBM showed rimmed vacuoles, autoaggressive inflammatory exudates, and congophilic inclusions. IBM-2 presented additional mitochondrial dysfunction. IBM-4 and IBM-5 additionally displayed atrophic myofibers. C-1 had a clinically silent leukocytosis with C-reactive protein elevation at biopsy. C-2 showed no abnormalities on muscle biopsy or laboratory results. C-3 presented with status post statin therapy with transient slight creatine kinase elevation; however, the muscle histology was negative and there were no other abnormalities. The biopsied muscle is included, along with the number of laser-microdissected myofibers that, in each case, compose a total of 100,000 μm2 of myofiber area. The number of myofibers varies in the patients with sIBM given the high variability in myofiber diameter. Control fibers, in general, were smaller on average and, thus, more fibers had to be sampled from controls.
For patients, data are given as AIBM/NIBM.