Takahashi Seiya, Inoue Hiroyasu, Amano Shizuki, Shinohara Takahiro, Mori Sara, Onizawa Kaho, Nabeshima Yoko, Komuro Hiroyasu, Yasumoto Taro, Mitsuhashi Rihito, Watanabe Daishi, Komaba Kazuki, Futamura Akinori, Nogawa Satoshi, Kinno Ryuta
Department of Neurology, Showa University Fujigaoka Hospital, Yokohama, Kanagawa, Japan.
Department of Rehabilitation, Showa University Fujigaoka Hospital, Yokohama, Kanagawa, Japan.
Medicine (Baltimore). 2024 Dec 13;103(50):e40912. doi: 10.1097/MD.0000000000040912.
Anti-mitochondrial antibodies (AMA) M2-positive myositis can lead to severe respiratory failure. Traditional immunotherapies sometimes fail to address respiratory failure. Herein, this CARE-compliant case report described a patient with AMA-M2-positive myositis who recovered from ventilation with tracheostomy owing to immunotherapy-resistant respiratory failure to spontaneous breathing after modified lung volume recruitment (mLVR) therapy.
A 60-year-old man experienced general fatigue and shortness of breath. The patient had a notable degree of proximal muscle weakness. Blood test results revealed hyperCKemia. The serum AMA-M2 antibody was positive. A muscle magnetic resonance imaging revealed diffuse abnormal hyperintensities in both lower limb muscles. Needle electromyography demonstrated fibrillation and positive sharp waves at rest with early recruitment, suggesting myogenic changes. Progressive muscle weakness and clinical findings fulfilled the criteria for definite idiopathic inflammatory myopathy. We diagnosed him with AMA-M2-positive myositis. Notably, his thoracic mobility was decreased, resulting in CO2 narcosis, requiring ventilation. Two courses of intravenous methylprednisolone for 3 days followed by oral prednisolone for myopathy. However, his respiratory function remained compromised, resulting in a tracheostomy.
We diagnosed him with severe immunotherapy-resistant respiratory failure due to AMA-positive myositis that required ventilation and tracheostomy.
To address respiratory failure, this patient underwent the mLVR therapy using the LIC TRAINER (LT). The pressure was adjusted by the operator using a bag-valve mask (BVM), starting at 1500 mL and increasing it up to a maximum of 2500 mL, while avoiding excessive airway pressure.
Respiratory function was evaluated by objective measures using chest wall mobility, inspiratory capacity (IC), vital capacity (VC), tidal volume (TV), and subjective measures using the visual analogue scale (VAS). Both objective and subjective scales showed significant improvements. The patient was weaned off ventilation 48 days after the initiation of mLVR therapy.
The mLVR therapy is an effective respiratory rehabilitation for patients with weakened respiratory muscles or tracheostomy. It allows the intensity of treatment to be adjusted based on the patient's symptoms, making it both highly effective and safe. This case suggests that mLVR therapy may be effective in treating severe, immunotherapy-resistant respiratory failure in AMA-positive myositis.
抗线粒体抗体（AMA）M2阳性肌炎可导致严重呼吸衰竭。传统免疫疗法有时无法解决呼吸衰竭问题。在此，这份符合CARE标准的病例报告描述了一名AMA-M2阳性肌炎患者，该患者因免疫疗法抵抗性呼吸衰竭而接受气管切开通气，在改良肺容积复张（mLVR）治疗后恢复自主呼吸。
一名60岁男性出现全身乏力和呼吸急促。患者有明显程度的近端肌无力。血液检查结果显示肌酸激酶升高。血清AMA-M2抗体呈阳性。肌肉磁共振成像显示双下肢肌肉弥漫性异常高信号。针极肌电图显示静息时纤颤和正锐波，早期募集，提示肌源性改变。进行性肌无力和临床表现符合明确的特发性炎性肌病标准。我们诊断他为AMA-M2阳性肌炎。值得注意的是，他的胸廓活动度降低，导致二氧化碳麻醉，需要通气。给予两疗程静脉注射甲泼尼龙3天，随后口服泼尼松治疗肌病。然而，他的呼吸功能仍然受损，导致进行了气管切开术。
我们诊断他患有严重的免疫疗法抵抗性呼吸衰竭，原因是AMA阳性肌炎，需要通气和气管切开术。
为解决呼吸衰竭问题，该患者使用LIC TRAINER（LT）进行了mLVR治疗。操作人员使用球囊面罩（BVM）调节压力，起始压力为1500毫升，最大增加到2500毫升，同时避免气道压力过高。
通过使用胸壁活动度、吸气容量（IC）、肺活量（VC）、潮气量（TV）等客观指标以及视觉模拟量表（VAS）等主观指标评估呼吸功能。客观和主观量表均显示有显著改善。在开始mLVR治疗48天后，患者脱机。
mLVR治疗对于呼吸肌无力或气管切开的患者是一种有效的呼吸康复方法。它允许根据患者症状调整治疗强度，既高效又安全。该病例表明，mLVR治疗可能对治疗AMA阳性肌炎中严重的、免疫疗法抵抗性呼吸衰竭有效。
