UpToDate
Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate®

幼年型皮肌炎和多发性肌炎的诊断

Authors
Clare Hutchinson, MDCM, FRCPC
Brian M Feldman, MD, MSc, FRCPC
Section Editors
Thomas JA Lehman, MD
Marc C Patterson, MD, FRACP
Deputy Editor
Elizabeth TePas, MD, MS
Translators
常杏芝, 副教授

引言

幼年型皮肌炎(Juvenile dermatomyositis, JDM)和幼年型多发性肌炎(juvenile polymyositis, JPM)是罕见的儿童自身免疫性肌病。JDM的主要特征为毛细血管血管病变；而JPM则涉及到T细胞直接侵犯肌纤维，这与在成人多发性肌炎患者中所观察到的相似[1,2]。 (参见“成人皮肌炎与多发性肌炎的临床表现”)

在这两种疾病中JDM更为常见，约占儿童期特发性炎症性肌病的85%，而JPM仅占该病例的3%-6%。

JDM和JPM的诊断将总结在此，其发病机制、临床表现和治疗将在别处讨论。 (参见“幼年型皮肌炎和多发性肌炎的发病机制和临床表现”和“幼年型皮肌炎和多发性肌炎的治疗、并发症和预后”)

分类与诊断标准

在1975年，Bohan和Peter针对包括JDM在内的多种类型肌炎提出了一种分类模式和诊断标准[3]。他们提出的JDM诊断标准包括了以下所有表现[3]：

  • 对称性近端肌无力
  • 特征性皮肤改变，包括向阳性皮炎(上眼睑紫红色皮疹伴眶周水肿)和Gottron丘疹(指关节背侧面红色鳞屑性丘疹)(图片 1)
  • 血清中有一种肌酶水平升高
  • 肌电图(electromyography, EMG)显示去神经支配和肌肉病变。
  • 肌活检显示肌纤维坏死和炎症性浸润，如图A所示(图片 2)

                

Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: 2017-06 . | This topic last updated: 2016-02-08.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
References
Top
  1. Arahata K, Engel AG. Monoclonal antibody analysis of mononuclear cells in myopathies. I: Quantitation of subsets according to diagnosis and sites of accumulation and demonstration and counts of muscle fibers invaded by T cells. Ann Neurol 1984; 16:193.
  2. Banker BQ, Victor M. Dermatomyositis (systemic angiopathy) of childhood. Medicine (Baltimore) 1966; 45:261.
  3. Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med 1975; 292:344.
  4. Brown VE, Pilkington CA, Feldman BM, et al. An international consensus survey of the diagnostic criteria for juvenile dermatomyositis (JDM). Rheumatology (Oxford) 2006; 45:990.
  5. Schmeling H, Stephens S, Goia C, et al. Nailfold capillary density is importantly associated over time with muscle and skin disease activity in juvenile dermatomyositis. Rheumatology (Oxford) 2011; 50:885.
  6. Rider LG, Koziol D, Giannini EH, et al. Validation of manual muscle testing and a subset of eight muscles for adult and juvenile idiopathic inflammatory myopathies. Arthritis Care Res (Hoboken) 2010; 62:465.
  7. Manual Muscle Testing Procedures for MMT8 Testing. http://www.niehs.nih.gov/research/resources/assets/docs/mmt8_grading_and_testing_procedures_for_the_abbreviated_8_muscle_groups.pdf (Accessed on July 22, 2013).
  8. Harris-Love MO, Shrader JA, Koziol D, et al. Distribution and severity of weakness among patients with polymyositis, dermatomyositis and juvenile dermatomyositis. Rheumatology (Oxford) 2009; 48:134.
  9. Rider LG, Giannini EH, Harris-Love M, et al. Defining Clinical Improvement in Adult and Juvenile Myositis. J Rheumatol 2003; 30:603.
  10. Lovell DJ, Lindsley CB, Rennebohm RM, et al. Development of validated disease activity and damage indices for the juvenile idiopathic inflammatory myopathies. II. The Childhood Myositis Assessment Scale (CMAS): a quantitative tool for the evaluation of muscle function. The Juvenile Dermatomyositis Disease Activity Collaborative Study Group. Arthritis Rheum 1999; 42:2213.
  11. Smith RL, Sundberg J, Shamiyah E, et al. Skin involvement in juvenile dermatomyositis is associated with loss of end row nailfold capillary loops. J Rheumatol 2004; 31:1644.
  12. Dolezalova P, Young SP, Bacon PA, Southwood TR. Nailfold capillary microscopy in healthy children and in childhood rheumatic diseases: a prospective single blind observational study. Ann Rheum Dis 2003; 62:444.
  13. Spencer-Green G, Schlesinger M, Bove KE, et al. Nailfold capillary abnormalities in childhood rheumatic diseases. J Pediatr 1983; 102:341.
  14. Christen-Zaech S, Seshadri R, Sundberg J, et al. Persistent association of nailfold capillaroscopy changes and skin involvement over thirty-six months with duration of untreated disease in patients with juvenile dermatomyositis. Arthritis Rheum 2008; 58:571.
  15. Pachman LM, Abbott K, Sinacore JM, et al. Duration of illness is an important variable for untreated children with juvenile dermatomyositis. J Pediatr 2006; 148:247.
  16. Guzmán J, Petty RE, Malleson PN. Monitoring disease activity in juvenile dermatomyositis: the role of von Willebrand factor and muscle enzymes. J Rheumatol 1994; 21:739.
  17. Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts). N Engl J Med 1975; 292:403.
  18. McCann LJ, Juggins AD, Maillard SM, et al. The Juvenile Dermatomyositis National Registry and Repository (UK and Ireland)--clinical characteristics of children recruited within the first 5 yr. Rheumatology (Oxford) 2006; 45:1255.
  19. Hernandez RJ, Sullivan DB, Chenevert TL, Keim DR. MR imaging in children with dermatomyositis: musculoskeletal findings and correlation with clinical and laboratory findings. AJR Am J Roentgenol 1993; 161:359.
  20. Park JH, Niermann KJ, Ryder NM, et al. Muscle abnormalities in juvenile dermatomyositis patients: P-31 magnetic resonance spectroscopy studies. Arthritis Rheum 2000; 43:2359.
  21. Tzaribachev N, Well C, Schedel J, Horger M. Whole-body MRI: a helpful diagnostic tool for juvenile dermatomyositis case report and review of the literature. Rheumatol Int 2009; 29:1511.
  22. Crowe WE, Bove KE, Levinson JE, Hilton PK. Clinical and pathogenetic implications of histopathology in childhood polydermatomyositis. Arthritis Rheum 1982; 25:126.
  23. Miles L, Bove KE, Lovell D, et al. Predictability of the clinical course of juvenile dermatomyositis based on initial muscle biopsy: a retrospective study of 72 patients. Arthritis Rheum 2007; 57:1183.
  24. Wedderburn LR, Varsani H, Li CK, et al. International consensus on a proposed score system for muscle biopsy evaluation in patients with juvenile dermatomyositis: a tool for potential use in clinical trials. Arthritis Rheum 2007; 57:1192.
  25. Haas RH, Dyck RF, Dubowitz V, Pepys MB. C-reactive protein in childhood dermatomyositis. Ann Rheum Dis 1982; 41:483.
  26. Bloom BJ, Tucker LB, Miller LC, Schaller JG. von Willebrand factor in juvenile dermatomyositis. J Rheumatol 1995; 22:320.
  27. Rider LG, Miller FW, Targoff IN, et al. A broadened spectrum of juvenile myositis. Myositis-specific autoantibodies in children. Arthritis Rheum 1994; 37:1534.
  28. Rider LG, Shah M, Mamyrova G, et al. The myositis autoantibody phenotypes of the juvenile idiopathic inflammatory myopathies. Medicine (Baltimore) 2013; 92:223.
  29. Compeyrot-Lacassagne S, Feldman BM. Inflammatory myopathies in children. Rheum Dis Clin North Am 2007; 33:525.