Barohn R J, Amato A A, Sahenk Z, Kissel J T, Mendell J R
Department of Neurology, University of Texas Southwestern Medical Center, Dallas 75235-8897, USA.
Neurology. 1995 Jul;45(7):1302-4. doi: 10.1212/wnl.45.7.1302.
We treated eight patients who had inclusion body myositis (IBM) with oral prednisone therapy, and we performed muscle biopsies before and after treatment. We documented the patients' clinical response to therapy and changes in serum CK. Although the serum CK level fell, muscle strength worsened after prednisone treatment. In addition, while inflammation decreased in the muscle biopsy specimens, the number of vacuolated and amyloid-positive fibers increased after oral prednisone therapy. These observations indicate that the inflammatory response in IBM may play a secondary role in the pathogenesis of IBM. The unique findings of intracellular amyloid deposits and rimmed vacuoles distinguishing IBM from other inflammatory myopathies, and recognition that suppression of inflammation has no effect on the clinical course, suggest that IBM may represent a degenerative muscle disorder.
我们对8例包涵体肌炎（IBM）患者进行了口服泼尼松治疗，并在治疗前后进行了肌肉活检。我们记录了患者对治疗的临床反应以及血清肌酸激酶（CK）的变化。尽管血清CK水平下降，但泼尼松治疗后肌肉力量恶化。此外，虽然肌肉活检标本中的炎症减少，但口服泼尼松治疗后空泡化和淀粉样蛋白阳性纤维的数量增加。这些观察结果表明，IBM中的炎症反应可能在IBM的发病机制中起次要作用。细胞内淀粉样蛋白沉积和镶边空泡的独特发现将IBM与其他炎症性肌病区分开来，并且认识到炎症抑制对临床病程没有影响，这表明IBM可能是一种退行性肌肉疾病。
