末梢神経・筋サルコイドーシス

Sarcoid Peripheral Neuropathy and Myopathy: A Diagnostic and Therapeutic Challenge

末梢神経，筋サルコイドーシスは非乾酪性類上皮細胞肉芽腫が脳神経と脊髄神経，骨格筋に出現する疾患である。画像検査を含め，全身検索と他疾患の除外が重要で，確定診断には非乾酪性類上皮細胞肉芽腫の証明が必須である。いずれも治療はステロイド薬が第一選択ではあるが，複数の免疫抑制薬，TNF-α阻害薬を併用しながら長期の治療を要することが多い。治療薬のランダム化比較試験に乏しく，治療法の確立が望まれる。

Abstract

Sarcoidosis is an idiopathic granulomatous multi-organ disease, primarily affecting the respiratory system, eyes, and skin, with less involvement in peripheral neurons and muscles. Sarcoid peripheral neuropathy encompasses cranial and spinal nerve impairment. Muscle involvement is often asymptomatic and revealed through imaging. Symptomatic muscle involvement is categorized into three clinical types: nodular myopathy, acute myopathy, and chronic myopathy. The identification of noncaseating granulomas in peripheral nerves or muscles, coupled with the exclusion of other diseases, is essential for establishing a definitive diagnosis of sarcoid peripheral neuropathy and myopathy. Sarcoid neuropathy and myopathy are typically managed with high-dose corticosteroids, immunosuppressants, or a combination of both. In recent times, the use of TNF-alpha inhibitors has notably increased. However, these conditions often exhibit resistance to treatment and may necessitate prolonged therapeutic interventions. Therefore, comprehensive examinations should be conducted before considering immunotherapy. Due to the rarity of these conditions, research on manifestation-specific treatments is lacking, and standard treatments for sarcoid neuropathy and myopathy have not been established. Additional treatment options for sarcoid neuropathy and myopathy are expected to become available in the future.