title: Pipestem capillaries in necrotizing myopathy revisited.
abstract: Pipestem-capillaries in necrotizing myopathy, have been reported as a feature of a distinct type of myopathy. Here, we analyze four muscle biopsy specimens from patients exhibiting endomysial fibrosis associated with pipestem capillaries using histological and electronmicroscopic techniques. However, only one case displayed all of the originally described features, including necrotic fibres, capillary thickening and lack of a significant lymphocytic inflammation, while one case exhibited striking capillary pathology with minimal necrosis and absence of inflammation, and the other two cases were accompanied by additional pathological features. These data support the existence of a microangiopathy with pipestem capillaries as a characteristic and distinct histopathological pattern, and indicate that it occurs in the context of a variety of muscular disorders broader than initially suspected. Furthermore, we show that the pipestem-capillary associated decrease in fibre size is at least in part a result of hypoxic changes.
authors.affiliation: Institute of Pathology, Charité-Universitätsmedizin, 10117 Berlin, Germany.
authors.forename: Nicolas W J
authors.initials: NW
authors.lastname: Schröder
authors.forename: Hans-Hilmar
authors.initials: HH
authors.lastname: Goebel
authors.forename: Almuth
authors.initials: A
authors.lastname: Brandis
authors.forename: Axel-Michael
authors.initials: AM
authors.lastname: Ladhoff
authors.forename: Frank L
authors.initials: FL
authors.lastname: Heppner
authors.forename: Werner
authors.initials: W
authors.lastname: Stenzel
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D002196:Capillaries / Q000473:pathology*; D015938:Complement Membrane Attack Complex / Q000378:metabolism; D003402:Creatine Kinase / Q000378:metabolism; D005260:Female; D006801:Humans; D051795:Hypoxia-Inducible Factor 1, alpha Subunit / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000098:blood supply* / Q000378:metabolism / Q000473:pathology*; D009135:Muscular Diseases / Q000473:pathology*; D009336:Necrosis
chemical_list: D015938:Complement Membrane Attack Complex; C497442:HIF1A protein, human; D051795:Hypoxia-Inducible Factor 1, alpha Subunit; D003402:Creatine Kinase