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NCT Number: NCT04118725

Muscular Respiratory Involvement and Systemic Sclerosis

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.

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Key information

Conditions

Systemic Sclerosis Connective Tissue Diseases Diaphragm Defect Diaphragmatic Electromyography Muscle Weakness Muscular Diseases Muscular Weakness Musculoskeletal Diseases Nervous System Diseases Neurologic Manifestations Neuromuscular Manifestations Pathologic Processes Pathological Conditions, Signs and Symptoms Pulmonary Function Test Respiration Disorders Respiratory Insufficiency Respiratory Tract Diseases Scleroderma, Systemic Signs and Symptoms Skin Diseases Skin and Connective Tissue Diseases

Age range

18 year and older

Sex eligibility

All sexes

Study type

Interventional

Phase

Not applicable

Primary location

Chu de Poitiers

Poitiers, France

Who can participate

Healthy volunteers accepted: No

Only the study team can determine whether someone qualifies for participation.

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

Treatment and study plan

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)

Primary outcomes

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

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

    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

Secondary outcomes

  1. 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

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

    Proportion of ScS patients with diaphragmatic involvement confirmed by EMG (latency >8.5ms and amplitudes <0.5mV by neck electric phrenic stimulation and/or Pdi <20cmH2O after magnetic stimulation) compared to proportion of ScS patients with maxIP below definite z-score and/or lying/sitting LVC ratio <75%

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

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

    Proportion of ScS patients with diaphragmatic involvement confirmed by EMG (latency >8.5ms and amplitudes <0.5mV by neck electric phrenic stimulation and/or Pdi <20cmH2O after magnetic stimulation) compared to proportion of ScS patients with dyspnea (SADOUL ≥2) or without dyspnea

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

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

    Proportion of ScS patients with diaphragmatic involvement confirmed by EMG (latency >8.5ms and amplitudes <0.5mV by neck electric phrenic stimulation and/or Pdi <20cmH2O after magnetic stimulation) and proportion of ScS patients with dyspnea (SADOUL ≥2)

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

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

    Proportion of ScS patients with diaphragmatic involvement confirmed by EMG (latency >8.5ms and amplitudes <0.5mV by neck electric phrenic stimulation and/or Pdi <20cmH2O after magnetic stimulation) and : age in years ; proportion of female; proportion of limited cutaneous or diffuse cutaneous or sine scleroderma ; ScS duration in years, proportion of anti-centromere or anti-Scl70 or anti-ARNpolymeraseIII or "others" autoantibody; proportion of digital tip ulcerations; proportion of subcutaneous calcifications; proportion of arthritis; proportion of arterial pulmonary hypertension; proportion of interstitial lung disease; proportion of renal crisis; proportion of inflammatory myopathy; proportion of immunosuppressant use; modified Rodnan skin score (median, ranging from 0-51)); interlabial length in centimeter; proportion of telangiectasia; proportion of cardiac insufficiency (left and/or right heart); creatine phosphokinase level in UI/mL ; PO2 in mmHg; PCO2 in mmHg

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

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

    Proportion of ScS patients with diaphragmatic involvement confirmed by EMG (latency >8.5ms and amplitudes <0.5mV by neck electric phrenic stimulation and/or Pdi <20cmH2O after magnetic stimulation) and proportion of patients with maxIP below definite z-score and/or lying/sitting LVC ratio < 75%)

  6. Correlation between diaphragmatic involvement confirmed by EMG and and/or Pdi and quality of life and functional disability

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

    Proportion of ScS patients with diaphragmatic involvement confirmed by EMG (latency >8.5ms and amplitudes <0.5mV by neck electric phrenic stimulation and/or Pdi <20cmH2O after magnetic stimulation) and total score in the Functional Assessment of Chronic Illness Therapy (FACIT)-Dyspnea-short form (ranging from 0 to 30; higher values represent a worse outcome); physical summary score (mean) from the short form health survey 36 (SF-36) (French version 2.0) ranging from 0 to 100 (higher values represent a worse outcome) ; mental summary score (mean) from the short form health survey 36 (SF-36) (French version 2.0) ranging from 0 to 100 (higher values represent a worse outcome

Sponsors and collaborators

Lead sponsor

Poitiers University Hospital

Other

Registry information

Official study title

Assessment of Muscular Respiratory Involvement in Systemic Sclerosis

Acronym: SIROCO

Important dates

Study start
2019
Primary completion
2021
Study completion
2021
First posted
Oct 8, 2019
Registry last updated
Sep 29, 2021

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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