Clinical trial NCT04118725: Muscular Respiratory Involvement and Systemic Sclerosis
Official title: Assessment of Muscular Respiratory Involvement in Systemic Sclerosis
Study type: INTERVENTIONAL
Overall status: COMPLETED
Phase: NA
Conditions: Systemic Sclerosis
Diaphragm Defect
Respiratory Insufficiency
Pulmonary Function Test
Diaphragmatic Electromyography
Muscular Weakness
Lead sponsor: Poitiers University Hospital
Sponsor class: OTHER
First posted: 2019-10-08
Enrollment: 52
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Allocation: NA
Intervention model: SINGLE_GROUP
Primary purpose: SCREENING

Brief summary: Dyspnea in systemic sclerosis (ScS) constitute a major factor of functional disability.

Intensity of dyspnea is sometimes discordant with objectives data from cardiopulmonary involvements, suggesting unknown additional factors.

Diffuse fibrosing myopathy of bad prognosis have been reported in ScS.To now, muscular respiratory involvement has not been evaluated in ScS. Therefore, ScS patients (with or without dyspnea) could have underlying respiratory muscular involvement not detected by current standard of care with pulmonary function tests (PFT).

This project is the first, to the best of our knowledge, to assess frequency of respiratory muscular involvement in ScS and to evaluate a screening strategy of this involvement.


Eligibility criteria: Inclusion Criteria:

* Age ≥18 years-old
* ScS fulfilling ACR/EULAR 2013 classification criteria and followed in the department of internal medicine of Poitiers University Hospital
* Written informatory consent

Exclusion Criteria:

* Individuals under tutorship or guardianship
* Individuals with reinforced protection: minors, judiciary or administrative decision
* No affiliation to social insurance
* Pregnancy or breastfeeding
* Associated neuromuscular disease (myasthenia, degenerative diseases)
* Bedridden individuals or with health condition not allowing realization or interpretation of PFT and/or EMG (acute cardiac and/or respiratory failure, pneumopathy)
* Individuals with pacemaker and implanted pumps, especially sensing dispositives, as defibrillators ( relative contre-indication to EMG with magnetic stimulation)
* Individuals with contre-indication to gastroesophageal probe: INR \>4, oesophageal varices, suspected cardio-facial fracture, oesophageal and/or facial obstacle

Primary outcomes:
measure: Frequency of diaphragmatic involvement among ScS patients with dyspnea and/or with suspected respiratory muscular involvement on pulmonary function tests (PFT)
timeFrame: 4 months maximum (maximum time between PFT realization and date to obtain EMG/Pdi)
Secondary outcomes:
measure: Sensitivity, specificity, positive and negative predictive value of pulmonary function tests compared to diaphragmatic involvement confirmed by EMG and Pdi among ScS patients with suspected pulmonary muscular involvement on PFT
timeFrame: 4 months maximum (maximum time between PFT realization and date to obtain EMG/Pdi)
measure: Sensitivity, specificity, positive and negative predictive value of dyspnea compared to diaphragmatic involvement confirmed by EMG and Pdi
timeFrame: 4 months maximum (maximum time between PFT realization and date to obtain EMG/Pdi)
measure: Correlation between diaphragmatic involvement confirmed by EMG and and/or Pdi and dyspnea
timeFrame: 4 months maximum (maximum time between PFT realization and date to obtain EMG/Pdi)
measure: Correlation between diaphragmatic involvement confirmed by EMG and and/or Pdi and general and ScS-related factors
timeFrame: 4 months maximum (maximum time between PFT realization and date to obtain EMG/Pdi)
measure: Correlation between diaphragmatic involvement confirmed by EMG and and/or Pdi and suspected muscular respiratory on pulmonary function test
timeFrame: 4 months maximum (maximum time between PFT realization and date to obtain EMG/Pdi)
measure: Correlation between diaphragmatic involvement confirmed by EMG and and/or Pdi and quality of life and functional disability
timeFrame: 4 months maximum (maximum time between PFT realization and date to obtain EMG/Pdi)
Other outcomes:
Locations:
city: Poitiers
country: France
facility: Chu de Poitiers
Study officials:
References: