Distal inflammatory myopathy: Unusual presentation of polymyositis or new entity?
| Autor: | Serge Herson, Bruno Eymard, Brigitte Ranque, Pascal Laforêt, Dalia Dimitri, Thierry Maisonobe, Olivier Benveniste, Anthony Behin, Emmanuel Fournier, Jean-Marie Mussini, Christian Pagnoux, Odile Dubourg, Thomas Papo |
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| Rok vydání: | 2008 |
| Předmět: |
Male
Weakness Pathology medicine.medical_specialty Polymyositis Inflammatory myopathy Antigens CD medicine Humans Muscle Skeletal Genetics (clinical) Myositis Retrospective Studies Muscle biopsy medicine.diagnostic_test Electromyography business.industry Rimmed vacuoles Middle Aged Dermatomyositis medicine.disease Neurology Pediatrics Perinatology and Child Health Female Neurology (clinical) medicine.symptom Inclusion body myositis business |
| Zdroj: | Neuromuscular Disorders. 18:493-500 |
| ISSN: | 0960-8966 |
| Popis: | New classification of idiopathic inflammatory myopathy (IIM) defined three major entities, polymyositis (PM), dermatomyositis (DM) and sporadic inclusion body myositis (s-IBM). We report the clinical, electrophysiological and pathological characteristics of three patients with a rare form of IIM not fulfilling the diagnostic criteria for any of these three major entities. The three patients presented with a subacute, distal asymmetrical weakness in upper limbs. Muscle biopsy showed an active myositis, with necrosis and regeneration, T cell infiltrates with invasion of non-necrotic fibers, without rimmed vacuoles, and diffuse major histocompatibility complex-I (MHC-I) immunostaining in muscle fibers. All patients responded to immunosuppressive agents. Seven others cases were identified in the literature. It is important to recognize this atypical presentation as it seems to respond to immunosuppressive agents. |
| Databáze: | OpenAIRE |
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