Overview
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.
Description
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 (MIP measured with Powerbreathe KH2 and RP Check), spirometry (FEV1, FVC, FIVC, IRV), diaphragm ultrasound (mobility and thickness fraction), six-minute walk test (6MWT), fatigue assessment (Borg scale, Fatigue Severity Scale), and quality of life (SF-36). 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 EULAR/ACR 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.
Eligibility
Inclusion Criteria:
- Diagnosis of inflammatory myopathy according to EULAR/ACR 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
Exclusion Criteria:
- 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