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- Dezsi, LiviaSzegedi Tudomanyegyet, Altalonos Orvostudomanyi Kar, Szent Gyorgyi Albert Klinikai Kozpont, Neurol Klin, Szeged, Hungary (author)
Inclusion body myositis - a rarely recognized disorder
- Article/chapterHungarian2013
Publisher, publication year, extent ...
- Budapest, Hungary :Literatura Medica Kiado,2013
- printrdacarrier
Numbers
- LIBRIS-ID:oai:DiVA.org:liu-92707
- https://urn.kb.se/resolve?urn=urn:nbn:se:liu:diva-92707URI
Supplementary language notes
- Language:Hungarian
- Summary in:English
Part of subdatabase
- SwePubSwePub
Classification
Notes
- Inclusion body myositis is the most common disabling inflammatory myopathy in the elderly. It is more frequent in men and after the age of 50 years. Inflammatory and degenerative features coexist. There is a T-cell mediated autoimmunity driven by in situ clonally expanded cytotoxic CD8-positive T-cells invading non-necrotic muscle fibres expressing MHC-I antigen. The hallmarks of degeneration are the deposition of protein aggregates and the formation of vesicles. The course of the disease is slow and the diagnosis is usually set after several years. The muscle weakness and wasting is assymetric, affecting predominantly distal muscles of the upper extremity and proximal muscles of the legs. The signs and clinical course can be characteristic, but the diagnosis is established by muscle biopsy. less thanbrgreater than less thanbrgreater thanThere is currently no evidence based effective treatment for sIBM. Prednisone, azathioprine, methotrexate, cyclosporine and IFN-beta failed. Oxandrolon did not improve symptoms. Treatment with intravenous immunglobuline (IVIG) induced in some patients a transient improvement of swallowing and of muscle strenght, but the overall study results were negative. less thanbrgreater than less thanbrgreater thanA T-cell depleting monoclonal antibody (alemtuzumab), in a small uncontrolled study slowed down disease progression for a six-month period. Repeated muscle biopsies showed the reduction of T-cells in the muscle and the suppression of some degeneration associated molecules. An effective therapeutic mean should act on both aspects of the pathomechanism, on the inflammatory and the degenerative processes as well.
Subject headings and genre
- MEDICIN OCH HÄLSOVETENSKAP Medicinska och farmaceutiska grundvetenskaper Fysiologi hsv//swe
- MEDICAL AND HEALTH SCIENCES Basic Medicine Physiology hsv//eng
- sIBM
- inflammation
- degenerative process
- amyloid precursor protein
- endoplasmic reticulum
- immunosuppressive treatment
- MEDICINE
- MEDICIN
Added entries (persons, corporate bodies, meetings, titles ...)
- Danielsson, OlofLinköpings universitet,Institutionen för klinisk och experimentell medicin,Hälsouniversitetet(Swepub:liu)oloda25 (author)
- Gati, IstvanLinköpings universitet,Institutionen för klinisk och experimentell medicin,Hälsouniversitetet(Swepub:liu)istga26 (author)
- Timea Varga, EdinaSzegedi Tudomanyegyet, Altalonos Orvostudomanyi Kar, Szent Gyorgyi Albert Klinikai Kozpont, Neurol Klin, Szeged, Hungary (author)
- Vecsei, LaszloSzegedi Tudomanyegyet, Altalonos Orvostudomanyi Kar, Szent Gyorgyi Albert Klinikai Kozpont, Neurol Klin, Szeged, Hungary (author)
- Szegedi Tudomanyegyet, Altalonos Orvostudomanyi Kar, Szent Gyorgyi Albert Klinikai Kozpont, Neurol Klin, Szeged, HungaryInstitutionen för klinisk och experimentell medicin (creator_code:org_t)
Related titles
- In:Ideggyogyaszati Szemle - Clinical NeuroscienceBudapest, Hungary : Literatura Medica Kiado66:3-4, s. 89-1010019-1442
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