Churg-Strauss 症候群：病例報告

Churg-Strauss Syndrome: A Case Report

李婉嘉(Wan-Chia Lee) ； 周正亮(Cheng-Liang Chou) ； 潘柏榮(Po-Zong Pan) ； 劉作仁(Tcho-Jen Liu) ； 詹瑞棋(Rai-Chi Chan)

《台灣復健醫學雜誌》 32卷3期 (2004/09) Pp. 155-161

https://doi.org/10.6315/2004.32(3)07

摘要

Churg-Strauss syndrome (CSS) is a rare systemic vasculitis of unknown cause, occurring in patients with asthma and accompanied by hypereosinophilia. Pathologically, CSS is characterized by necrotising eosinophilic vasculitis which may involve multiple organ systems, commonly including the lungs, skin, and peripheral nervous system. Corticosteroids are the first-line therapy, with cyclophosphamide as adjuvant therapy. The overall prognosis is good.
We report a case of CSS with the clinical pattern of asthma, hypereosinophlilia (＞10%), pulmonary infiltration, and mononeuritis multiplex. Progressive weakness and numbness in the bilateral hands and lower limbs, with associated walking inability, remained a concern despite steroid treatment. The patient underwent rehabilitation therapy including strengthening exercises, balance training, ambulation training, activities of daily living (ADL) training, and hand function training. We used a Functional Independence Measure motor score and muscle power as a follow-up index. Five months later, she showed improvement in left ankle dorsiflexion muscle power and was able to walk independently with a simple cane. There was a significant improvement in ADL independence and skill. The intractable neuropathic pain was treated successfully by a topical application of capsaicin.
In this article, we review the clinical features, diagnosis criteria, and treatment options of CSS. Atrophy and weakness of the limbs, paresthesia, and neuropathic pain result in the functional impairment of daily living and ambulation disability. Thus, in addition to pharmacological treatment, aggressive rehabilitation intervention is essential to improve the patient's functional independence of daily living and life quality.

關鍵字

Churg-Strauss症候群 ； 復健 ； 辣椒膏

並列摘要

Churg-Strauss syndrome (CSS) is a rare systemic vasculitis of unknown cause, occurring in patients with asthma and accompanied by hypereosinophilia. Pathologically, CSS is characterized by necrotising eosinophilic vasculitis which may involve multiple organ systems, commonly including the lungs, skin, and peripheral nervous system. Corticosteroids are the first-line therapy, with cyclophosphamide as adjuvant therapy. The overall prognosis is good.
We report a case of CSS with the clinical pattern of asthma, hypereosinophlilia (＞10%), pulmonary infiltration, and mononeuritis multiplex. Progressive weakness and numbness in the bilateral hands and lower limbs, with associated walking inability, remained a concern despite steroid treatment. The patient underwent rehabilitation therapy including strengthening exercises, balance training, ambulation training, activities of daily living (ADL) training, and hand function training. We used a Functional Independence Measure motor score and muscle power as a follow-up index. Five months later, she showed improvement in left ankle dorsiflexion muscle power and was able to walk independently with a simple cane. There was a significant improvement in ADL independence and skill. The intractable neuropathic pain was treated successfully by a topical application of capsaicin.
In this article, we review the clinical features, diagnosis criteria, and treatment options of CSS. Atrophy and weakness of the limbs, paresthesia, and neuropathic pain result in the functional impairment of daily living and ambulation disability. Thus, in addition to pharmacological treatment, aggressive rehabilitation intervention is essential to improve the patient's functional independence of daily living and life quality.

並列關鍵字

Churg-Strauss syndrome ； rehabilitation ； capsaicin