دورية أكاديمية
[Juvenile-onset anti-nuclear matrix protein 2 (NXP-2) antibody-positive dermatomyositis with joint contractures before manifestation of myositis: a case report].
| العنوان: | [Juvenile-onset anti-nuclear matrix protein 2 (NXP-2) antibody-positive dermatomyositis with joint contractures before manifestation of myositis: a case report]. |
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| المؤلفون: | Miura E; Department of Neurology, Nagaoka Red Cross Hospital., Taneda T; Department of Neurology, Nagaoka Red Cross Hospital., Umeda Y; Department of Neurology, Nagaoka Red Cross Hospital., Umeda M; Department of Neurology, Nagaoka Red Cross Hospital., Oyake M; Department of Neurology, Nagaoka Red Cross Hospital., Matsushita T; Department of Dermatology, Kanazawa University., Nishino I; Department of Neuromuscular Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry (NCNP)., Fujita N; Department of Neurology, Nagaoka Red Cross Hospital. |
| المصدر: | Rinsho shinkeigaku = Clinical neurology [Rinsho Shinkeigaku] 2024 Jun 27; Vol. 64 (6), pp. 417-421. Date of Electronic Publication: 2024 May 25. |
| نوع المنشور: | Journal Article; Case Reports; English Abstract |
| اللغة: | Japanese |
| بيانات الدورية: | Publisher: Nihon Shinkei Gakkai Country of Publication: Japan NLM ID: 0417466 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1882-0654 (Electronic) Linking ISSN: 0009918X NLM ISO Abbreviation: Rinsho Shinkeigaku Subsets: MEDLINE |
| أسماء مطبوعة: | Publication: April 1963-: Tōkyō : Nihon Shinkei Gakkai Original Publication: Tōkyō : Nihon Rinshō Shinkei Gakkai, 1960- |
| مواضيع طبية MeSH: | Dermatomyositis*/complications , Dermatomyositis*/immunology , Dermatomyositis*/diagnosis , Dermatomyositis*/drug therapy , Autoantibodies*/blood , Contracture*/etiology , Contracture*/diagnosis , Biomarkers*/blood , Pulse Therapy, Drug*, Humans ; Male ; Young Adult ; Methylprednisolone/administration & dosage ; Diagnosis, Differential ; RNA-Binding Proteins/immunology ; Nuclear Proteins/immunology ; Adult ; Adenosine Triphosphatases ; DNA-Binding Proteins ; Transcription Factors |
| مستخلص: | A 23-year-old man was admitted to our hospital with a one-year history of muscle weakness and atrophy. He had noticed contractures of the fingers of both hands from the age of 18. Examination revealed a skin rash including heliotrope rash and Gottron's sign, joint contractures in the extremities, dysphagia, extensive muscle weakness and marked muscle atrophy. The serum creatine kinase level was 272 IU/l and muscle biopsy showed typical perifascicular atrophy but little lymphocyte invasion. There was no interstitial pneumonia or malignancy, but muscle tendons showed elevated CT values suggesting calcification or fibrosis. Anti-nuclear matrix protein 2 (NXP-2) antibody-positive dermatomyositis was diagnosed on the basis of the serum antibody level. Methylprednisolone pulse therapy ameliorated the skin rash and bulbar palsy, but muscle weakness, atrophy and joint contractures were resistant to the treatment. There have been no previous reports of young adults with anti-NXP-2 antibody-positive dermatomyositis in whom joint contracture became evident as early as 4 years beforehand, which is a important feature for differential diagnosis of dermatomyositis. |
| فهرسة مساهمة: | Keywords: anti-nuclear matrix protein 2 (NXP-2) antibody; dermatomyositis; joint contracture; juvenile onset |
| المشرفين على المادة: | 0 (Autoantibodies) 0 (Biomarkers) EC 3.6.1.- (MORC3 protein, human) X4W7ZR7023 (Methylprednisolone) 0 (RNA-Binding Proteins) 0 (Nuclear Proteins) 0 (nuclear matrix protein 2) EC 3.6.1.- (Adenosine Triphosphatases) 0 (DNA-Binding Proteins) 0 (Transcription Factors) |
| تواريخ الأحداث: | Date Created: 20240526 Date Completed: 20240626 Latest Revision: 20240626 |
| رمز التحديث: | 20240627 |
| DOI: | 10.5692/clinicalneurol.cn-001970 |
| PMID: | 38797685 |
| قاعدة البيانات: | MEDLINE |
| تدمد: | 1882-0654 |
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| DOI: | 10.5692/clinicalneurol.cn-001970 |