Inclusion body myositis – pathomechanism and lessons from genetics
Murnyák Balázs,
Bodoki Levente,
Vincze Melinda,
Griger Zoltán,
Csonka Tamás,
Szepesi Rita,
Kurucz Andrea,
Dankó Katalin,
Hortobágyi Tibor
Affiliations
Murnyák Balázs
Division of Neuropathology,
Institute of Pathology
Bodoki Levente
Institute
of Internal Medicine, Third Department of Internal Medicine,
Division of Clinical Immunology
Vincze Melinda
Institute
of Internal Medicine, Third Department of Internal Medicine,
Division of Clinical Immunology
Griger Zoltán
Institute
of Internal Medicine, Third Department of Internal Medicine,
Division of Clinical Immunology
Csonka Tamás
Division of Neuropathology,
Institute of Pathology
Szepesi Rita
Department of Neurology, University of Debrecen,
Faculty of Medicine, Debrecen, Hungary
Kurucz Andrea
Division of Neuropathology,
Institute of Pathology
Dankó Katalin
Institute
of Internal Medicine, Third Department of Internal Medicine,
Division of Clinical Immunology
Hortobágyi Tibor
University of Debrecen,
Faculty of Medicine, Institute of Pathology, Division of Neuropathology,
4032 Debrecen, Nagyerdei krt. 98. Tel.: + 36 52 255-248;
e-mail: [email protected]
Inclusion body myositis is a rare, late-onset myopathy. Both inflammatory and myodegenerative features play an important role in their pathogenesis. Overlapping clinicopathological entities are the familial inclusion body myopathies with or without dementia. These myopathies share several clinical and pathological features with the sporadic inflammatory disease. Therefore, better understanding of the genetic basis and pathomechanism of these rare familial cases may advance our knowledge and enable more effective treatment options in sporadic IBM, which is currently considered a relentlessly progressive incurable disease.
