inclusion_body_myositis
Table of Contents
inclusion body myositis (IBM)
see also:
introduction
- rare but increasingly prevalent (with the aging population) chronic inflammatory myopathy
- slowly progressive weakness and wasting of both distal and proximal muscles, of both arms and legs, and unlike polymyositis (PM) and dermatomyositis, responds poorly to corticosteroids and other immunosuppressants
3 main forms
sporadic (sIBM)
- mixed autoimmune and degenerative processes
- incidence increases with age and symptoms usually begin after 50 years of age
- 50 cases per million in those aged over 50 years (compared with 15 cases per million in all ages)
- the most common acquired muscle disorder seen in people over 50
- 20% have onset before age 50yrs
- weakness comes on slowly (over months or years)
- slightly more common in men than women
- may become unable to perform daily living activities and most require assistive devices within 5 to 10 years of symptom onset
- may cause potentially fatal dysphagia
- no effective treatment exists
familial IBM
- occurs in siblings but not passed to children
hereditary (hIBM)
- heterogeneous group of very rare hereditary conditions
- IBM2:
- mainly Iranian Jew ethnicity
- if the quadriceps are spared but the hamstrings and iliopsoas are severely affected in a person between ages of 20-40, it is very likely IBM2 will be at the top of the differential diagnosis.
- see wikipedia
Dx
- raised creatinine kinase (CK)
- muscle biopsy:
- inclusion bodies, rimmed vacuoles and accumulation of aberrant proteins similar to those found in senile plaques of Alzheimer's disease
inclusion_body_myositis.txt · Last modified: 2013/09/27 06:53 by 127.0.0.1