Inclusion body myositis

Inclusion body myositis (IBM) (maIou'saItIs) (sometimes called sporadic inclusion body myositis, sIBM) is the most common inflammatory muscle disease in older adults. 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. IBM is often confused with an entirely different class of diseases, called hereditary inclusion body myopathies (hIBM). The "M" in hIBM is an abbreviation for "myopathy" while the "M" in IBM is for "myositis". 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. sIBM is a rare disease, with a prevalence ranging from 1 to 71 individuals per million. 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. Patients may become unable to perform activities of daily living and most require assistive devices within 5 to 10 years of symptom onset. sIBM does not significantly affect life expectancy, although death related to malnutrition and respiratory failure can occur. The risk of serious injury due to falls is increased. There is no effective treatment for the disease as of 2019. IBM stands for "inclusion body myositis: not "inclusion body myopathy." The 'inclusion body' refers to a histological finding of rimmed vacuoles in muscle tissue. However, IBM does not refer to the collection of diseases that feature these inclusion bodies. It refers to a specific disease entity. Multiple genetic diseases that feature inclusion bodies have been grouped into "hereditary inclusion body myopathies (hIBM).

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Concepts associés (6)

Polymyositis

Polymyositis (PM) is a type of chronic inflammation of the muscles (inflammatory myopathy) related to dermatomyositis and inclusion body myositis. Its name means 'inflammation of many muscles' (poly- + myos- + -itis). The inflammation of polymyositis is mainly found in the endomysial layer of skeletal muscle, whereas dermatomyositis is characterized primarily by inflammation of the perimysial layer of skeletal muscles. The hallmark of polymyositis is weakness and/or loss of muscle mass in the proximal musculature, as well as flexion of the neck and torso.

Muscle squelettique

Les muscles squelettiques sont les muscles sous contrôle volontaire du système nerveux central. Le corps humain comprend environ 570 muscles présents chez tous les individus sains. Leur corps contient des vaisseaux sanguins, des nerfs, des organes sensoriels, du tissu conjonctif commun, et des cellules musculaires. En microscopie photonique (ou optique), ils présentent une double striation longitudinale et transversale. La science du muscle est la myologie. Les myoblastes sont les cellules précurseurs des muscles.