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Inclusion body myositis - a rarely recognized disorder
Szegedi Tudomanyegyet, Altalonos Orvostudomanyi Kar, Szent Gyorgyi Albert Klinikai Kozpont, Neurol Klin, Szeged, Hungary.
Linköping University, Department of Clinical and Experimental Medicine. Linköping University, Faculty of Health Sciences.
Linköping University, Department of Clinical and Experimental Medicine. Linköping University, Faculty of Health Sciences.
Szegedi Tudomanyegyet, Altalonos Orvostudomanyi Kar, Szent Gyorgyi Albert Klinikai Kozpont, Neurol Klin, Szeged, Hungary.
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2013 (Hungarian)In: Ideggyogyaszati Szemle - Clinical Neuroscience, ISSN 0019-1442, Vol. 66, no 3-4, 89-101 p.Article, review/survey (Refereed) Published
Abstract [en]

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.

Place, publisher, year, edition, pages
Budapest, Hungary: Literatura Medica Kiado , 2013. Vol. 66, no 3-4, 89-101 p.
Keyword [en]
sIBM, inflammation, degenerative process, amyloid precursor protein, endoplasmic reticulum, immunosuppressive treatment
National Category
Physiology
Identifiers
URN: urn:nbn:se:liu:diva-92707ISI: 000317633400002OAI: oai:DiVA.org:liu-92707DiVA: diva2:621649
Available from: 2013-05-16 Created: 2013-05-16 Last updated: 2015-08-27Bibliographically approved

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Danielsson, OlofGati, Istvan

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