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Brain. 2015 Sep;138(Pt 9):2485-92. doi: 10.1093/brain/awv192. Epub 2015 Jul 21.

Anti-Jo-1 antibody-positive patients show a characteristic necrotizing perifascicular myositis.

Author information

1
1 Département de Pathologie, Institut de Pathologie, Centre Hospitalier Universitaire, Grenoble, France.
2
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.
3
4 INSERM: CIMI-Paris, INSERM UMRS-1135, UPMC, Paris.
4
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.
5
6 Laboratoire de Neuropathologie, Centre de Référence Maladies Neuro-Musculaires Paris Est, AP-HP, Hôpital Pitié-Salpêtrière, Paris, France.
6
5 Département de Médecine Interne, Hôpital Dijon, Dijon, France.
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3 Department of Neuropathology, Charité-Universitätsmedizin, Berlin, Germany.
8
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.

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.

KEYWORDS:

anti-synthetase; dermatomyositis; idiopathic inflammatory myopathies; necrotizing myopathies; polymyositis

PMID:
26198592
DOI:
10.1093/brain/awv192
[Indexed for MEDLINE]

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