Personalized Home Respiratory Rehabilitation Program for Subjects With Systemic Sclerosis With Early Lung Disease

Study Overview
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Brief Summary
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Systematic sclerosis (SSc) is a potentially severe disease characterized by various visceral involvements including lung. The investigators hypothesize that a respiratory rehabilitation program specifically designed for people with systematic sclerosis with early lung disease could help to decrease respiratory deficiencies, improve aerobic capacity and prevent activity limitations and participation restrictions. Before testing the effectiveness of such a program, a pilot study is needed to assess its feasibility and optimize its content. Participants will have 1 supervised session in the outpatient rehabilitation department. Each patient will then perform the home personalized exercises program for 3 months. The feasibility of the program will be assessed at 3 months using patients' adherence to the program (assessed by the number of lost to follow-up, the number of questionnaires not completed, the amount of aerobic activity and the amount of home personalized exercises, treatment burden, adverse effects and quality of life.

Detailed Description
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SSc is responsible for a reduced life expectancy. The prognosis depends on the presence of severe visceral damage and more particularly on the presence of interstitial lung damage, pulmonary arterial hypertension or specific cardiac damage which represent the 3 main causes of mortality in SSc. The importance of rehabilitation in SSc has been confirmed by the latest INSERM Collective Expertise 2019). The objectives of functional rehabilitation in SSc are to prevent and/or reduce the specific (cutaneous, oral, cardiorespiratory, musculoskeletal) and non-specific (exercise deconditioning, fatigue, anxiety and depression) impairments frequently characterizing the evolution of the disease. Based on the latest practice recommendations for the diagnosis and treatment of idiopathic pulmonary fibrosis, people with a confirmed diagnosis of idiopathic pulmonary fibrosis and significant exercise and activity limitations should follow a respiratory rehabilitation program. The value of a specific respiratory rehabilitation program for people with SSc is then a matter of interest. A pilot study is needed to assess the feasibility and optimize the content of a rehabilitation program. The investigators aimed to study the feasibility of a personalized home-based respiratory rehabilitation program in people with SSc with early lung disease.

Official Title
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Personalized Home Respiratory Rehabilitation Program for Subjects With Systemic Sclerosis With Early Lung Disease: Pilot Feasibility Study

Conditions
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Systemic Sclerosis

Intervention / Treatment
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* Other: Rehabilitation


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Systemic sclerosis according to ACR/EULAR 2013 criteria Disease duration < 7 years Lung involvement, with FCV > 70% on PFT Exclusion Criteria: Inability to understand French Pregnancy or breastfeeding Arterial pulmonary hypertension > 35 mmHg and unexplained dyspnoea or arterial pulmonary hypertension > 40 mmHg Major musculoskeletal impairment incompatible with physical activity other pulmonary disease decreasing FCV Pathological EKG Oxygen saturation at rest or during physical activity < 90% FCV < 70%

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 65 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Other
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Home based exercises<br>1 supervised session in the outpatient rehabilitation department with respiratory, aerobic and muscles strengthening exercises, followed by 3 months of self-manage personalized home exercises program. Cardiac frequency and adherence to exercise will be monitored using an activity tracker (Garmin© watch). | Other: Rehabilitation<br>* 1 supervised session of rehabilitation, including a cardiac stress test (to define maximal intensity for aerobic activities), aerobic activity with interval training of moderate and progressive intensity (walking), and respiratory exercise using a volumetric spirometer, and strengthening exercises for lower and upper limbs. 3 months of self-managed home exercises. Patients will be asked to use a tracker activity when performing home exercises to self-monitor cardiac frequency and to assess their adherence.<br>* Other names: Personalized home exercises program;|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Number of lost to follow up patients | Adherence to treatment | 3 months | 
| Number of uncompleted questionnaires | Adherence to treatment | 3 months | 
| Amount of aerobic work recorded using a connected watch | Adherence to treatment / Amount of aerobic work recorded using a connected watch with heart rate monitor | 3 months | 
| Amount of exercises self-reported in a logbook | Adherence to treatment | 3 months | 
| Exercise Adherence Rating Scale (EARS) questionnaire | Adherence to treatment / Amount of exercises | 3 months | 
| Self-reported adherence | Adherence to treatment | 3 months | 
| Exercise Adherence Rating Scale (EARS) questionnaire | Adherence to treatment | 3 months | 
| Treatment burden assess by the Exercise Therapy Burden Questionnaire (ETBQ) | Adherence to treatment / Burden of program | 3 months | 
| Check-list to report side effects | Adherence to treatment | 3 months | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Lung volume | Pulmonary Function Testing (PFT) - Respiratory function / Variation between base line and 3 months | Day of inclusion | 
| Lung volume | Pulmonary Function Testing (PFT) - Respiratory function / Variation between base line and 3 months | 3 months | 
| Diffusing capacity | Pulmonary Function Testing (PFT) - Respiratory function / Variation between base line and 3 months | Day of inclusion | 
| Diffusing capacity | Pulmonary Function Testing (PFT) - Respiratory function / Variation between base line and 3 months | 3 months | 
| Maximal oxygen consumption during a 6 minute walking test | Aerobic capacity / Variation between base line and 3 months | Day of inclusion | 
| Maximal oxygen consumption during a 6 minute walking test | Aerobic capacity / Variation between base line and 3 months | 3 months | 
| Saint Georges hospital questionnaire | Activity limitations specific to respiratory function / Variation between base line and 3 months | Day of inclusion | 
| Saint Georges hospital questionnaire | Activity limitations specific to respiratory function / Variation between base line and 3 months | 3 months | 
| Health Assessment Questionnaire (HAQ) | Overall activity limitations / Variation between base line and 3 months | Day of inclusion | 
| Health Assessment Questionnaire (HAQ) | Overall activity limitations / Variation between base line and 3 months | 3 months | 
| 12-Items short form survey questionnaire (SF-12) | Quality of life / Variation between base line and 3 months | Day of inclusion | 
| 12-Items short form survey questionnaire (SF-12) | Quality of life / Variation between base line and 3 months | 3 months | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Systemic Sclerosis, Lung disease, Rehabilitation, Respiratory deficiencies, Feasibility study