Inclusion body myopathy
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Inclusion body myopathy
Pathology
Bone disorders
Osteomyelitis
Bone tumors
Chondrosarcoma
Osteochondroma
Achondroplasia
Arthrogryposis
Cleidocranial dysplasia
Club foot
Craniosynostosis
Flat feet
Genu valgum
Genu varum
Osteogenesis imperfecta
Pectus excavatum
Pigeon toe
Lordosis, kyphosis, and scoliosis
Osteomalacia and rickets
Osteopetrosis
Osteoporosis
Osteosclerosis
Paget disease of bone
Musculoskeletal injuries and trauma
Degenerative disc disease
Sciatica
Spinal disc herniation
Spondylolisthesis
Spondylolysis
Achilles tendon rupture
Anterior cruciate ligament injury
Iliotibial band syndrome
Meniscus tear
Patellar tendon rupture
Patellofemoral pain syndrome
Sprained ankle
Unhappy triad
Compartment syndrome
Rhabdomyolysis
Carpal tunnel syndrome
Erb-Duchenne palsy
Klumpke paralysis
Sciatica
Thoracic outlet syndrome
Ulnar claw
Winged scapula
Carpal tunnel syndrome
Dislocated shoulder
Erb-Duchenne palsy
Klumpke paralysis
Radial head subluxation (Nursemaid elbow)
Rotator cuff tear
Thoracic outlet syndrome
Ulnar claw
Winged scapula
Musculoskeletal system pathology review
Back pain: Pathology review
Bone disorders: Pathology review
Bone tumors: Pathology review
Gout and pseudogout: Pathology review
Muscular dystrophies and mitochondrial myopathies: Pathology review
Myalgias and myositis: Pathology review
Neuromuscular junction disorders: Pathology review
Pediatric musculoskeletal disorders: Pathology review
Rheumatoid arthritis and osteoarthritis: Pathology review
Scleroderma: Pathology review
Seronegative and septic arthritis: Pathology review
Sjogren syndrome: Pathology review
Systemic lupus erythematosus (SLE): Pathology review
Assessments
Inclusion body myopathy
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Inclusion body myopathy
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Content Reviewers
In inclusion body myopathy, “myopathy” refers to muscle disease and “inclusion body” refers to the presence of inclusions, or vacuoles, formed by clumps of protein that collect within the muscle fibers.
There’s a sporadic form, sporadic meaning that it strikes at random, which is the most common and is also called inclusion body myositis - because it causes muscle inflammation.
There’s also a rare hereditary form, that causes no muscle inflammation.
Normally, the cells of the immune system are ready to spot and destroy anything foreign that could cause the body harm.
To help with this, most cells in the body have a set of proteins that come together to form a major histocompatibility complex, or MHC, class I proteins which sits on the surface of their cell membrane.
These surface proteins act kind of like a serving platter, presenting molecules from within the cell for the immune system, so that it can have a way of performing ongoing surveillance.
Normally the MHC class I proteins serves up a normal harmless molecule from the cell - a self-antigen, and there’s no response.
But if a cell is invaded by a pathogen like a virus, then viral proteins are served upon on the MHC class I proteins.
When these viral antigens are displayed on the cell surface, it sparks an immune response.
Specifically, a type of T-lymphocyte, called a CD8+ T-cell or a cytotoxic T-cell, will bind to the antigen presented by the MHC class I proteins.
If the cytotoxic T-cell binds strongly, than the antigen is recognized as foreign, and the cytotoxic T-cell secretes inflammatory molecules and enzymes - like perforin and granzymes.
Perforin is able to form holes in the infected cell and that allows the granzymes to enter the cell.
Once inside, the granzymes induce apoptosis, or programmed cell death - which destroys the cell.
And as if that weren’t enough, the cytotoxic T-cells have a protein called Fas ligand on their surface.
Fas ligand binds to a protein called Fas on the surface of the infected cell.
Sources
- "Robbins Basic Pathology" Elsevier (2017)
- "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
- "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
- "CURRENT Medical Diagnosis and Treatment 2020" McGraw-Hill Education / Medical (2019)
- "GNE myopathy: from clinics and genetics to pathology and research strategies" Orphanet Journal of Rare Diseases (2018)
- "Hereditary inclusion-body myopathies" Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease (2015)
- "GNE Myopathy: Etiology, Diagnosis, and Therapeutic Challenges" Neurotherapeutics (2018)
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