Systemic Sclerosis
Conditions
Keywords
Systemic Sclerosis, Lung disease, Rehabilitation, Respiratory deficiencies, Feasibility study
Brief summary
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
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.
Interventions
OTHERRehabilitation
* 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.
Sponsors
Assistance Publique - Hôpitaux de Paris
URC-CIC Paris Descartes Necker Cochin
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
OTHER
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to 65 Years
Healthy volunteers
No
Inclusion criteria
* Systemic sclerosis according to ACR/EULAR 2013 criteria * 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%
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Number of lost to follow up patients | 3 months | Adherence to treatment |
| Number of uncompleted questionnaires | 3 months | Adherence to treatment |
| Amount of aerobic work recorded using a connected watch | 3 months | Adherence to treatment / Amount of aerobic work recorded using a connected watch with heart rate monitor |
| Amount of exercises self-reported in a logbook | 3 months | Adherence to treatment |
| Exercise Adherence Rating Scale (EARS) questionnaire | 3 months | Adherence to treatment / Amount of exercises |
| Self-reported adherence | 3 months | Adherence to treatment |
| Treatment burden assess by the Exercise Therapy Burden Questionnaire (ETBQ) | 3 months | Adherence to treatment / Burden of program |
| Check-list to report side effects | 3 months | Adherence to treatment |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Lung volume | Day of inclusion | Pulmonary Function Testing (PFT) - Respiratory function / Variation between base line and 3 months |
| Diffusing capacity | Day of inclusion | Pulmonary Function Testing (PFT) - Respiratory function / Variation between base line and 3 months |
| Maximal oxygen consumption during a 6 minute walking test | Day of inclusion | Aerobic capacity / Variation between base line and 3 months |
| Saint Georges hospital questionnaire | Day of inclusion | Activity limitations specific to respiratory function / Variation between base line and 3 months |
| Health Assessment Questionnaire (HAQ) | Day of inclusion | Overall activity limitations / Variation between base line and 3 months |
| 12-Items short form survey questionnaire (SF-12) | Day of inclusion | Quality of life / Variation between base line and 3 months |
Countries
France
Contacts
PRINCIPAL_INVESTIGATORCamille DASTE, MD
Cochin Hospital, Assistance Publique des Hôpitaux de Paris (AP-HP)
STUDY_DIRECTORChristelle NGUYEN, MD, PhD
Cochin Hospital, Assistance Publique des Hôpitaux de Paris (AP-HP)
Outcome results
None listed