title: Anti-Jo-1 antibody-positive patients show a characteristic necrotizing perifascicular myositis.
abstract: Idiopathic inflammatory myopathies can be classified as polymyositis, dermatomyositis, immune-mediated necrotizing myopathy, sporadic inclusion body myositis or non-specific myositis. Anti-Jo-1 antibody-positive patients are assigned to either polymyositis or dermatomyositis suggesting overlapping pathological features. We aimed to determine if anti-Jo-1 antibody-positive myopathy has a specific morphological phenotype. In a series of 53 muscle biopsies of anti-Jo-1 antibody-positive patients, relevant descriptive criteria defining a characteristic morphological pattern were identified. They were tested in a second series of anti-Jo-1 antibody-positive patients and compared to 63 biopsies from patients suffering from other idiopathic inflammatory myopathies. In anti-Jo-1 antibody-positive patients, necrotic fibres, which strongly clustered in perifascicular regions, were frequently observed. Sarcolemmal complement deposition was detected specifically in perifascicular areas. Inflammation was mainly located in the perimysium and around vessels in 90.6%. Perimysial fragmentation was observed in 90% of cases. Major histocompatibility complex class I staining was diffusely positive, with a perifascicular reinforcement. Multivariate analysis showed that criteria defining perifascicular pathology: perifascicular necrosis, atrophy, and perimysial fragmentation allow the distinction of anti-Jo-1 antibody-positive patients, among patients suffering from other idiopathic inflammatory myopathies. Anti-Jo-1 antibody-positive patients displayed perifascicular necrosis, whereas dermatomyositis patients exhibited perifascicular atrophy.
authors.affiliation: 1 Département de Pathologie, Institut de Pathologie, Centre Hospitalier Universitaire, Grenoble, France.
authors.forename: Lénaig
authors.initials: L
authors.lastname: Mescam-Mancini
authors.affiliation: 2 Département de Médecine Interne et Immunologie Clinique, Centre de Référence Maladies Neuro-Musculaires Paris Est, Assistance Public - Hôpitaux de Paris (AP-HP), DHU I2B, Université Pierre et Marie Curie (UPMC), INSERM UMRS 974, Hôpital Pitié-Salpêtrière, Paris, France 3 Department of Neuropathology, Charité-Universitätsmedizin, Berlin, Germany.
authors.forename: Yves
authors.initials: Y
authors.lastname: Allenbach
authors.affiliation: 2 Département de Médecine Interne et Immunologie Clinique, Centre de Référence Maladies Neuro-Musculaires Paris Est, Assistance Public - Hôpitaux de Paris (AP-HP), DHU I2B, Université Pierre et Marie Curie (UPMC), INSERM UMRS 974, Hôpital Pitié-Salpêtrière, Paris, France 3 Department of Neuropathology, Charité-Universitätsmedizin, Berlin, Germany.
authors.forename: Baptiste
authors.initials: B
authors.lastname: Hervier
authors.affiliation: 4 INSERM: CIMI-Paris, INSERM UMRS-1135, UPMC, Paris.
authors.forename: Hervé
authors.initials: H
authors.lastname: Devilliers
authors.affiliation: 2 Département de Médecine Interne et Immunologie Clinique, Centre de Référence Maladies Neuro-Musculaires Paris Est, Assistance Public - Hôpitaux de Paris (AP-HP), DHU I2B, Université Pierre et Marie Curie (UPMC), INSERM UMRS 974, Hôpital Pitié-Salpêtrière, Paris, France.
authors.forename: Kuberaka
authors.initials: K
authors.lastname: Mariampillay
authors.affiliation: 6 Laboratoire de Neuropathologie, Centre de Référence Maladies Neuro-Musculaires Paris Est, AP-HP, Hôpital Pitié-Salpêtrière, Paris, France.
authors.forename: Odile
authors.initials: O
authors.lastname: Dubourg
authors.affiliation: 5 Département de Médecine Interne, Hôpital Dijon, Dijon, France.
authors.forename: Thierry
authors.initials: T
authors.lastname: Maisonobe
authors.affiliation: 6 Laboratoire de Neuropathologie, Centre de Référence Maladies Neuro-Musculaires Paris Est, AP-HP, Hôpital Pitié-Salpêtrière, Paris, France.
authors.forename: Romain
authors.initials: R
authors.lastname: Gherardi
authors.affiliation: 1 Département de Pathologie, Institut de Pathologie, Centre Hospitalier Universitaire, Grenoble, France.
authors.forename: Paulette
authors.initials: P
authors.lastname: Mezin
authors.affiliation: 3 Department of Neuropathology, Charité-Universitätsmedizin, Berlin, Germany.
authors.forename: Corinna
authors.initials: C
authors.lastname: Preusse
authors.affiliation: 3 Department of Neuropathology, Charité-Universitätsmedizin, Berlin, Germany.
authors.forename: Werner
authors.initials: W
authors.lastname: Stenzel
authors.affiliation: 2 Département de Médecine Interne et Immunologie Clinique, Centre de Référence Maladies Neuro-Musculaires Paris Est, Assistance Public - Hôpitaux de Paris (AP-HP), DHU I2B, Université Pierre et Marie Curie (UPMC), INSERM UMRS 974, Hôpital Pitié-Salpêtrière, Paris, France yves.allenbach@psl.aphp.fr.
authors.forename: Olivier
authors.initials: O
authors.lastname: Benveniste
mesh_terms: D000328:Adult; D000974:Antibodies, Antinuclear / Q000276:immunology*; D001323:Autoantibodies / Q000097:blood*; D003882:Dermatomyositis / Q000473:pathology*; D005260:Female; D006801:Humans; D008025:Ligases / Q000276:immunology; D008297:Male; D046529:Microscopy, Electron, Transmission; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000473:pathology* / Q000648:ultrastructure; D009220:Myositis / Q000097:blood / Q000276:immunology / Q000473:pathology; D018979:Myositis, Inclusion Body / Q000473:pathology*; D012189:Retrospective Studies; D018709:Statistics, Nonparametric
chemical_list: D000974:Antibodies, Antinuclear; D001323:Autoantibodies; C046750:Jo-1 antibody; D008025:Ligases
keywords: anti-synthetase; dermatomyositis; idiopathic inflammatory myopathies; necrotizing myopathies; polymyositis