包涵体肌炎的治疗与预后
- Author
- Marc L Miller, MD
Marc L Miller, MD
- Clinical Assistant Professor of Medicine
- Tufts University School of Medicine
- Section Editors
- Ira N Targoff, MD
Ira N Targoff, MD
- Section Editor — Muscle Disease
- Professor of Medicine, Section of Rheumatology
- University of Oklahoma Health Sciences Center
- Jeremy M Shefner, MD, PhD
Jeremy M Shefner, MD, PhD
- Section Editor — Neuromuscular Disease
- Professor and Chair of Neurology, Barrow Neurological Institute
- Professor of Neurology, University of Arizona, Phoenix
- Clinical Professor of Neurology, Creighton University
- Deputy Editor
- Monica Ramirez Curtis, MD, MPH
Monica Ramirez Curtis, MD, MPH
- Deputy Editor — Rheumatology
- Instructor of Medicine, Part-time
- Harvard Medical School
- Translators
- 谈文峰, 副主任医师，副教授
谈文峰, 副主任医师，副教授
- 江苏省人民医院风湿免疫科
引言
散发性包涵体肌炎(inclusion body myositis, IBM)与多发性肌炎和皮肌炎一起，被归类为特发性炎性肌病的一种。然而，尽管在组织学上有某些相似之处，但在临床病理表现、治疗和预后上，IBM与这两种疾病有明显的不同(表 1)。 (参见“成人皮肌炎与多发性肌炎的临床表现”和“成人皮肌炎和多发性肌炎的初始治疗”和“成人复发性及难治性皮肌炎和多发性肌炎的治疗”)
IBM的治疗和预后总结在此。其临床表现和诊断参见其他专题。 (参见“包涵体肌炎的临床表现与诊断”)
治疗
与皮肌炎和多发性肌炎等其他炎性肌病相反，标准免疫抑制治疗对IBM相对无效，且肌力对糖皮质激素和其他药物治疗即使有反应也很轻微。如果要在一项安慰剂对照研究中证明针对IBM治疗的明显疗效，据估计将需要纳入200例受试者进行为期6个月的研究，或纳入100例受试者进行为期1年的试验[1]。当分析下文中关于治疗有效性的数据时，应牢记上述估计值。 (参见下文‘具体治疗’)
治疗方法 — IBM患者的肌力很少会改善，导致一些医师认为治疗的目标为防止肌力进一步恶化，而非改善肌力[2]。但一个问题在于，IBM常在症状出现后数年才被诊断出。此时可能已有明显的肌肉损伤，即使可阻止疾病进程，但肌力也难以改善。
另一个局限是，持续性炎症可能并不是持续性或进行性肌无力的原因。例如，糖皮质激素治疗前、后进行的连续肌肉活检已表明，患者出现炎症消退和炎性细胞侵入的肌纤维数量减少，但无临床改善[3]。此外，随着时间的推移，空泡纤维的数量和淀粉样沉积的程度均增加。 (参见“包涵体肌炎的临床表现与诊断”)
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