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Inclusion body myositis (IBM) (/ m aɪ oʊ ˈ s aɪ t ɪ s /) (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 ...
Inclusion body myositis, a more common and non-hereditary form. Valosin-containing protein (VCP); mutations in VCP cause multisystem proteinopathy (MSP) which can present (among others) as a rare form of inclusion body myopathy.
Inclusion bodies are aggregates of specific types of protein found in neurons, and a number of tissue cells including red blood cells, bacteria, viruses, and plants. Inclusion bodies of aggregations of multiple proteins are also found in muscle cells affected by inclusion body myositis and hereditary inclusion body myopathy. [1]
It can also be associated with underlying cancer. The main classes of idiopathic inflammatory myopathy are polymyositis (PM), dermatomyositis (DM) (including juvenile, amyopathic, and sine-dermatitis form), inclusion-body myositis (IBM), immune-mediated necrotising myopathy (IMNM), and focal autoimmune myositis. [1]
Multisystem proteinopathy (MSP) is a dominantly inherited, pleiotropic, degenerative disorder of humans that can affect muscle, bone, and/or the central nervous system.MSP can manifest clinically as classical amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD), inclusion body myopathy (IBM), Paget's disease of bone (PDB), or as a combination of these disorders. [1]
Autoimmune - Autoimmune disease is an abnormal immune response to specific body protein or other biomolecular target, such as one of the muscles. The three main types of idiopathic myositis (known as inflammatory myopathies) that typically test positive for autoantibodies are dermatomyositis, polymyositis, and inclusion body myositis. [4]
Although they vary in particulars, polymyositis, dermatomyositis and inclusion body myositis are idiopathic inflammatory myopathies (IIM) [1] primarily characterized by chronic inflammation of human skeletal muscle tissue [2] that ultimately causes the necrosis of muscle cells. This degeneration leads to muscle tissue wasting, weakness and ...
HLA-DR3 has been consistently observed at high frequencies in inclusion body myositis in caucasians. [37] DR3 was found to correlate also with Jo-1 antibody presence. [38] Studies of sporadic inclusion body myositis indicate association with A1:DQ2 haplotype. [39]
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