The Effect of Inspiratory Muscle Training on the Functional Status of Patients With Inflammatory Myopathy
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 发起方
- 入组人数
- 33
- 试验地点
- 2
- 主要终点
- Change in maximal inspiratory pressure (MIP)
研究概览
简要总结
Inflammatory myopathies are rare autoimmune diseases leading to progressive muscle weakness, often including the respiratory muscles. This study aims to investigate whether inspiratory muscle training (IMT) using a threshold device can improve functional status in patients with inflammatory myopathy. Thirty-three patients will undergo a 3-month home-based IMT program with progressive resistance. Functional capacity, inspiratory muscle strength, lung function, diaphragmatic mobility, fatigue, and quality of life will be assessed at baseline and during follow-up. The primary hypothesis is that IMT will enhance inspiratory muscle strength and translate into better functional performance and quality of life.
详细描述
Idiopathic inflammatory myopathies (IIM) are rare autoimmune disorders characterized by progressive, symmetrical proximal muscle weakness, frequently affecting the respiratory muscles. Diaphragmatic dysfunction and reduced inspiratory muscle strength contribute to impaired ventilation, fatigue, and reduced exercise tolerance. While exercise training has proven beneficial in patients with various neuromuscular conditions, evidence regarding inspiratory muscle training (IMT) in IIM is lacking.
This pilot interventional study will prospectively evaluate the impact of a 3-month IMT program on respiratory and functional outcomes in patients with IIM. The intervention consists of daily inspiratory resistance training (30 breaths/day, 7 days/week) using the Threshold IMT device (Philips Respironics, USA). Training intensity is set at 30% of maximal inspiratory pressure (MIP) and adjusted progressively based on patient-reported exertion. Participants receive individualized instruction, follow-up phone consultations every 2 weeks, and record adherence in exercise diaries.
Assessments include inspiratory muscle strength (measured with Powerbreathe KH2), spirometry (Forced Expiratory Volume in 1 Second, Forced Vital Capacity, Forced Inspiratory Vital Capacity, Inspiratory Reserve Volume), diaphragm ultrasound (mobility and thickness fraction), six-minute walk test (6MWT), fatigue assessment (Borg Rating of Perceived Exertion Scale, Fatigue Severity Scale), and quality of life (36-Item Short Form Health Survey). Outcomes will be measured 3 months before training, at baseline, after 3 months of training, and during extended follow-up (up to 6-12 months). The primary endpoint is change in MIP. Secondary endpoints include pulmonary function parameters, diaphragmatic function, exercise tolerance, fatigue, and health-related quality of life.
The study will be conducted in the Neurology Clinic of the Medical University of Warsaw (WUM). A total of 33 patients with IIM, diagnosed according to European Alliance of Associations for Rheumatology / American College of Rheumatology criteria, will be recruited. Inclusion requires age ≥18 and written informed consent. Exclusion criteria include cognitive impairment, acute respiratory infection, prior respiratory training, pneumothorax, pulmonary hypertension, or contraindications to IMT. This trial is expected to provide the first structured evidence on the role of IMT in improving functional status in patients with inflammatory myopathy, potentially guiding individualized rehabilitation strategies for this patient population.
研究设计
- 研究类型
- Interventional
- 分配方式
- Na
- 干预模型
- Single Group
- 主要目的
- Supportive Care
- 盲法
- None
入排标准
- 年龄范围
- 18 Years 至 —（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Diagnosis of inflammatory myopathy according to European Alliance of Associations for Rheumatology / American College of Rheumatology criteria
- •Age ≥ 18 years
- •Stable clinical condition allowing participation in physical activity
- •Ability to understand and follow instructions
- •Written informed consent to participate in the study
排除标准
- •Cognitive impairment preventing proper exercise performance
- •Current respiratory tract infection
- •Previous respiratory muscle training within the last 12 months
- •History of spontaneous pneumothorax
- •Pulmonary hypertension
- •Tympanic membrane rupture or other middle ear pathology
- •Lack of voluntary and informed consent
研究组 & 干预措施
Inspiratory Muscle Training (IMT)
Patients with inflammatory myopathy will perform a 3-month inspiratory muscle training (IMT) program using a threshold device (Philips Respironics Threshold IMT). Training will be home-based, preceded by individual instruction, and monitored through exercise diaries and follow-up phone consultations. Assessments will be performed before training, after 3 months, and during follow-up visits.
干预措施: Inspiratory Muscle Training (IMT) (Behavioral)
结局指标
主要结局
Change in maximal inspiratory pressure (MIP)
时间窗: 3 months before training, baseline, 3 months (end of training), 6 months, 9 months, 12 months
Maximal inspiratory pressure (MIP) measured at the mouth using Powerbreathe KH2 devices. Results are expressed in cmH₂O. Higher values indicate greater inspiratory muscle strength.
次要结局
- Change in 6-Minute Walk Distance (6MWD)(3 months before training, baseline, 3 months (end of training), 6 months, 9 months, 12 months)
- Change in fatigue severity (Fatigue Severity Scale)(3 months before training, baseline, 3 months (end of training), 6 months, 9 months, 12 months)
- Change in diaphragm thickness(3 months before training, baseline, 3 months (end of training), 6 months, 9 months, 12 months)
- Change in quality of life (SF-36)(3 months before training, baseline, 3 months (end of training), 6 months, 9 months, 12 months)
- Change in Forced Vital Capacity (FVC)(3 months before training, baseline, 3 months (end of training), 6 months, 9 months, 12 months)
- Change in Forced Expiratory Volume in 1 Second (FEV1)(3 months before training, baseline, 3 months (end of training), 6 months, 9 months, 12 months)
- Change in 6-Minute Walk Distance (6-Minute Walk Test)(3 months before training, baseline, 3 months (end of training), 6 months, 9 months, 12 months)
- Change in perceived exertion (Borg Rating of Perceived Exertion)(3 months before training, baseline, 3 months (end of training), 6 months, 9 months, 12 months)
- Change in quality of life (36-Item Short Form Health Survey)(3 months before training, baseline, 3 months (end of training), 6 months, 9 months, 12 months)