Muscular Respiratory Involvement and Systemic Sclerosis

NCT04118725 | Completed

• 1 other identifier: 2019-A01047-50
• Study Type: interventional
• Target: 52
• Locations: 1 country
• Sites: 1

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.

Conditions

Systemic Sclerosis; Diaphragm Defect; Respiratory Insufficiency; Pulmonary Function Test; Diaphragmatic Electromyography; Muscular Weakness

Outcome Measures

Primary Outcomes (1)

• Frequency of diaphragmatic involvement among ScS patients with dyspnea and/or with suspected respiratory muscular involvement on pulmonary function tests (PFT)

  Number of patients with dyspnea (SADOUL ≥2) and/or with respiratory muscular involvement suspected on PFT (maximum inspiratory pressure (max IP) below definite z-score and/or lying/sitting low vital capacity (LVC) ratio \< 75%) having diaphragmatic muscular involvement confirmed by diaphragmatic electromyography (EMG) (latency \>8.5ms and amplitudes \<0.5mV) by neck electric phrenic stimulation and/or Pdi \<20cmH2O after magnetic stimulation

  4 months maximum (maximum time between PFT realization and date to obtain EMG/Pdi)

Secondary Outcomes (6)

• 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

  4 months maximum (maximum time between PFT realization and date to obtain EMG/Pdi)

• Sensitivity, specificity, positive and negative predictive value of dyspnea compared to diaphragmatic involvement confirmed by EMG and Pdi

  4 months maximum (maximum time between PFT realization and date to obtain EMG/Pdi)

• Correlation between diaphragmatic involvement confirmed by EMG and and/or Pdi and dyspnea

  4 months maximum (maximum time between PFT realization and date to obtain EMG/Pdi)

• Correlation between diaphragmatic involvement confirmed by EMG and and/or Pdi and general and ScS-related factors

  4 months maximum (maximum time between PFT realization and date to obtain EMG/Pdi)

• Correlation between diaphragmatic involvement confirmed by EMG and and/or Pdi and suspected muscular respiratory on pulmonary function test

  4 months maximum (maximum time between PFT realization and date to obtain EMG/Pdi)

Study Arms (1)

Muscular explorations

EXPERIMENTAL

Pulmonary function test and diaphragmatic electromyography

Diagnostic Test: Pulmonary function test

Interventions

Pulmonary function test DIAGNOSTIC_TEST

Diaphragmatic electromyography with measurement of transdiaphragmatic pressure in cases of dyspnea and/or abnormal pulmonary function test (maximum inspiratory pressure and/or lying/seating low vital capacity ratio)

Muscular explorations

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)

You may qualify if:

• 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

You may not qualify if:

• 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

Sponsors & Collaborators

• Poitiers University Hospital: lead

Study Sites (1)

Chu de Poitiers

Poitiers, France

Location

MeSH Terms

Conditions

Scleroderma, Systemic; Respiratory Insufficiency; Muscle Weakness

Interventions

Respiratory Function Tests

Condition Hierarchy (Ancestors)

Connective Tissue Diseases; Skin and Connective Tissue Diseases; Skin Diseases; Respiration Disorders; Respiratory Tract Diseases; Muscular Diseases; Musculoskeletal Diseases; Neuromuscular Manifestations; Neurologic Manifestations; Nervous System Diseases; Pathologic Processes; Pathological Conditions, Signs and Symptoms; Signs and Symptoms

Intervention Hierarchy (Ancestors)

Diagnostic Techniques, Respiratory System; Diagnostic Techniques and Procedures; Diagnosis

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: NA
• Masking: NONE
• Purpose: SCREENING
• Intervention Model: SINGLE GROUP
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: September 26, 2019
• First Posted: October 8, 2019
• Study Start: October 21, 2019
• Primary Completion: July 7, 2021
• Study Completion: July 7, 2021
• Last Updated: September 29, 2021

Record last verified: 2021-09

Locations

FR (1)