Zárványtestes myositis - Egy ritkán felismert betegség

Translated title of the contribution: Inclusion body myositis - A rarely recognized disorder

Lívia Dézsi, Olof Danielsson, István Gáti, Edina Tímea Varga, L. Vécsei

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

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. There is currently no evidence based effective treatment for sIBM. Prednisone, azathioprine, methotrexate, cyclosporine and IFN-β 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. A 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.

Original languageHungarian
Pages (from-to)89-101
Number of pages13
JournalIdeggyógyászati szemle
Volume66
Issue number3-4
Publication statusPublished - Mar 30 2013

Fingerprint

Inclusion Body Myositis
Muscles
T-Lymphocytes
Biopsy
Myositis
Muscle Weakness
Azathioprine
Deglutition
Prednisone
Autoimmunity
Methotrexate
Upper Extremity
Cyclosporine
Disease Progression
Leg
Therapeutics
Monoclonal Antibodies

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

Cite this

Dézsi, L., Danielsson, O., Gáti, I., Varga, E. T., & Vécsei, L. (2013). Zárványtestes myositis - Egy ritkán felismert betegség. Ideggyógyászati szemle, 66(3-4), 89-101.

Zárványtestes myositis - Egy ritkán felismert betegség. / Dézsi, Lívia; Danielsson, Olof; Gáti, István; Varga, Edina Tímea; Vécsei, L.

In: Ideggyógyászati szemle, Vol. 66, No. 3-4, 30.03.2013, p. 89-101.

Research output: Contribution to journalArticle

Dézsi, L, Danielsson, O, Gáti, I, Varga, ET & Vécsei, L 2013, 'Zárványtestes myositis - Egy ritkán felismert betegség', Ideggyógyászati szemle, vol. 66, no. 3-4, pp. 89-101.
Dézsi L, Danielsson O, Gáti I, Varga ET, Vécsei L. Zárványtestes myositis - Egy ritkán felismert betegség. Ideggyógyászati szemle. 2013 Mar 30;66(3-4):89-101.
Dézsi, Lívia ; Danielsson, Olof ; Gáti, István ; Varga, Edina Tímea ; Vécsei, L. / Zárványtestes myositis - Egy ritkán felismert betegség. In: Ideggyógyászati szemle. 2013 ; Vol. 66, No. 3-4. pp. 89-101.
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