NIHON SARUKOIDOSHISU / NIKUGESHUSHIKKAN (The Japanese journal of sarcoidosis and other granulomatous disorders )
Online ISSN : 1884-6122
Print ISSN : 1345-0565
ISSN-L : 1345-0565
Clinical Feature and Pathomechanism of Muscle Fiber Destruction in Muscular Sarcoidosis
Toshihide Kumamoto
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2008 Volume 28 Issue 1 Pages 25-31

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Abstract

The pathomechanism of muscle fiber destruction in muscular sarcoidosis, especially nodular type, may be caused by direct invasion of inflammatory cells rather than by mechanical compression or ischemia. The invasive cells consist of macrophages, epithelioid cells and lymphocytes, enter into muscle fibers during the process of granuloma formation. Furthermore, protease cathepsin B, m-calpain and ubiquitin-proteasome, which are derived from epithelioid cells and macrophages in granulomas, apparently play an important role in muscle fiber destruction. Muscular sarcoidosis manifesting acute myositis or chronic myopathy is discussed along with a review of the literature. Some patients with nodular muscular sarcoidosis extending to all limb muscles or trunk were reported. These patients, however, did not develop any muscle weakness and wasting for a long time, suggesting that the pathomechanism of muscle fiber destruction may be different in chronic myopathic form from that in nodular muscular sarcoidosis. Some muscular sarcoidosis presenting clinical features, especially progressive muscular weakness and wasting, of acute myositis or chronic myopathy are associated with autoimmune myopathies such as dermatomyositis, myositis-overlap syndrome, or myasthenia gravis.

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