title: Inclusion body myositis - pathomechanism and lessons from genetics.
abstract: 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.
authors.affiliation: Division of Neuropathology, Institute of Pathology.
authors.forename: Balázs
authors.initials: B
authors.lastname: Murnyák
authors.affiliation: Institute of Internal Medicine, Third Department of Internal Medicine, Division of Clinical Immunology.
authors.forename: Levente
authors.initials: L
authors.lastname: Bodoki
authors.affiliation: Institute of Internal Medicine, Third Department of Internal Medicine, Division of Clinical Immunology.
authors.forename: Melinda
authors.initials: M
authors.lastname: Vincze
authors.affiliation: Institute of Internal Medicine, Third Department of Internal Medicine, Division of Clinical Immunology.
authors.forename: Zoltán
authors.initials: Z
authors.lastname: Griger
authors.affiliation: Division of Neuropathology, Institute of Pathology.
authors.forename: Tamás
authors.initials: T
authors.lastname: Csonka
authors.affiliation: Department of Neurology, University of Debrecen, Faculty of Medicine, Debrecen, Hungary.
authors.forename: Rita
authors.initials: R
authors.lastname: Szepesi
authors.affiliation: Division of Neuropathology, Institute of Pathology.
authors.forename: Andrea
authors.initials: A
authors.lastname: Kurucz
authors.affiliation: Institute of Internal Medicine, Third Department of Internal Medicine, Division of Clinical Immunology.
authors.forename: Katalin
authors.initials: K
authors.lastname: Dankó
authors.affiliation: University of Debrecen, Faculty of Medicine, Institute of Pathology, Division of Neuropathology, 4032 Debrecen, Nagyerdei krt. 98. Tel.: + 36 52 255-248.
authors.forename: Tibor
authors.initials: T
authors.lastname: Hortobágyi
keywords: DES; GNE; IBMPFD; TDP-43; VCP; genetics; sporadic inclusion body myositis