Inclusion body myositis | |
---|---|
Other names | sIBM |
Specialty | Rheumatology, Neurology, Neuromuscular medicine |
Symptoms | Weakness |
Usual onset | Typically after age 45[1] |
Differential diagnosis | deconditioning, hereditary muscle diseases[1] |
Frequency | 5-71/100,000,000[1] |
Inclusion body myositis (IBM) (/maɪoʊˈsaɪtɪs/) (sometimes called sporadic inclusion body myositis, sIBM) is the most common inflammatory muscle disease in older adults.[2] The disease is characterized by slowly progressive weakness and wasting of both proximal muscles (located on or close to the torso) and distal muscles (close to hands or feet), most apparent in the finger flexors and knee extensors.[3] IBM is often confused with an entirely different class of diseases, called hereditary inclusion body myopathies (hIBM).[4][5] The "M" in hIBM is an abbreviation for "myopathy" while the "M" in IBM is for "myositis".[6] In IBM, two processes appear to occur in the muscles in parallel, one autoimmune and the other degenerative. Inflammation is evident from the invasion of muscle fibers by immune cells. Degeneration is characterized by the appearance of holes, deposits of abnormal proteins, and filamentous inclusions in the muscle fibers.[7] sIBM is a rare disease, with a prevalence ranging from 1 to 71 individuals per million.[8][9]
Weakness comes on slowly (over months to years) in an asymmetric manner and progresses steadily, leading to severe weakness and wasting of arm and leg muscles. IBM is more common in men than women.[10] Patients may become unable to perform activities of daily living and most require assistive devices within 5 to 10 years of symptom onset.[11] sIBM does not significantly affect life expectancy,[1] although death related to malnutrition and respiratory failure can occur.[12] The risk of serious injury due to falls is increased.[1] There is no effective treatment for the disease as of 2019.[1]
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