title: Basement membrane remodelling and segmental fibrosis in sporadic inclusion body myositis.
abstract: Sporadic inclusion body myositis (sIBM) is a debilitating idiopathic inflammatory myopathy. Little is known about the pathogenetic mechanisms that lead to myofiber degeneration. In the present study, we evaluated the integrity of the myofiber basement membrane in non-necrotic myofibers invaded by inflammatory infiltrates. We used 100 ten mum thick serial sections obtained from biopsies of 5 patients suffering from sIBM. Biopsies from 5 patients suffering from polymyositis served as controls. We performed sequential HE staining and immunolabeling using anti-CD68, -CD8, -merosin, -laminin alpha4 chain, and -collagen IV antibodies. In sIBM, we detected a total of 89 non-necrotic myofibers that were invaded by inflammatory cells. The invasive process and its sequelae were segmental in nature and included destruction of the myofiber basement membrane, and eventually, partial replacement by fibrosis of the invaded myofiber. In polymyositis, we found only two myofibers that were affected in this way. In sIBM, basement membrane remodelling and irreversible replacement by fibrosis of myofibers appear to represent the end result of a process in which the balance between injury and repair are disrupted.
authors.affiliation: Institute of Brain Research, University of Tübingen, Calwerstr. 3, D-72076 Tübingen, Germany.
authors.forename: K
authors.initials: K
authors.lastname: Doppler
authors.forename: M
authors.initials: M
authors.lastname: Mittelbronn
authors.forename: A
authors.initials: A
authors.lastname: Lindner
authors.forename: A
authors.initials: A
authors.lastname: Bornemann
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D015703:Antigens, CD / Q000378:metabolism; D015214:Antigens, Differentiation, Myelomonocytic / Q000378:metabolism; D001485:Basement Membrane / Q000276:immunology* / Q000378:metabolism / Q000473:pathology*; D016827:CD8 Antigens / Q000378:metabolism; D003094:Collagen / Q000378:metabolism; D003402:Creatine Kinase / Q000097:blood; D005260:Female; D005355:Fibrosis; D006801:Humans; D007150:Immunohistochemistry; D007797:Laminin / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D018485:Muscle Fibers, Skeletal / Q000276:immunology / Q000378:metabolism / Q000473:pathology*; D018979:Myositis, Inclusion Body / Q000276:immunology / Q000378:metabolism / Q000473:pathology*; D017285:Polymyositis / Q000473:pathology; D000097382:CD68 Molecule
chemical_list: D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D016827:CD8 Antigens; D003094:Collagen; D003402:Creatine Kinase; D007797:Laminin; C067980:CD68 antigen, human; D000097382:CD68 Molecule; C501369:LAMA4 protein, human