| Prevalence | 5-50 per million adults overall; up to 51 per million in adults over 50 years (Australia, highest reported) |
| Incidence | Approximately 2.5 per million per year (Sweden)[@comorbidities] |
| Age at onset | Mean approximately 64 years (range 45-80); extremely rare before age 45 |
| Sex | Male predominance with a male-to-female ratio of 2:1 to 3:1 |
| Ethnicity | More prevalent in Caucasian populations; lower reported rates in Asian and African populations |
| Mortality | 10-year survival 36-42% vs 59% in age-matched controls; mean age at death 79.3 years vs 83.6 years in controls[^6] |
| Disability | Progressive weakness leading to wheelchair dependence at a mean of approximately 10.5 years from onset |
| MHC class I upregulation | Sarcolemmal MHC-I expression is universally upregulated in IBM muscle fibers, even those without visible inflammatory infiltrates |
| [NF-κB](/entities/nf-kb) activation | The NF-κB signaling pathway is activated in IBM muscle fibers, driving both inflammatory gene expression and protein aggregation cascades |
| Anti-cN1A autoantibodies | Antibodies against cytosolic 5'-nucleotidase 1A are found in 33-76% of IBM patients with 87-100% specificity, supporting an autoimmune component[^8] |
| Immunosenescence | Highly differentiated T cells with features of immunosenescence (KLRG1+, CD57+, CD28-) dominate the IBM muscle infiltrate |
| p62/SQSTM1 and ubiquitin inclusions | p62 and ubiquitin-positive aggregates are a hallmark pathological finding |
| Databases | OMIMOrphanetClinicalTrialsPubMed |
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