Clinical Relevance of Thoracic Echography for the Early Diagnosis of Interstitial Lung Disease in Systemic Scleroderma - Pilot Study

Study Overview
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Brief Summary
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Diffuse interstitial lung disease (PID) is the leading cause of death in systemic scleroderma (SSc). Major progress has recently been made in its therapeutic management. Early diagnosis is essential to optimize this management. Current diagnostic techniques are based on high-resolution computed tomography on the thorax (HRCT) and pulmonary functional tests (PFT). However, these explorations have their limitations. Thus, there is a need for new techniques for a very early diagnosis of PID-SSc. Thoracic ultrasound (TUS) is an innovative, easily accessible, non-irradiating, inexpensive and painless tool. It is an emerging technique for the diagnosis of PID and has already proven its sensitivity for the detection of interstitial damage, as defined by HRCT. The main objective of the PRECOSS study is to describe the prevalence of an ultrasound interstitial syndrome in patients with SSc, free of PID-SSc (defined by the Goh criteria) detectable by HRCT.

Official Title
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Pertinence Clinique de l'échographie Thoracique Pour le Diagnostic précoce de la Pneumopathie Interstitielle Diffuse de la sclérodermie systémique - Etude Pilote

Conditions
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Systemic Sclerosis

Intervention / Treatment
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* Other: thoracic echography


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Age ≥ 18 years old Diagnosis of systemic scleroderma according to EULAR/ACR 2013 criteria Absence of diffuse interstitial lung disease proven by scanner on the day of inclusion Forced vital capacity ≥ 80% of the value predicted on the last respiratory function test performed. Carbon monoxide diffusion capacity corrected for hemoglobinemia ≥ 70% of the value predicted on the last respiratory function test performed. Patient capable of performing functional exploration during the year Affiliation to a social security system Patient who has given free and informed consent Exclusion Criteria: Any other connectivity associated to systemic sleroderma Any chronic pathologies, co-morbidities, history judged by the investigator as being likely to lead to complications for the patient and/or which may impact the results of functional exploration during exercise. All known fibrotic and/or obstructive respiratory pathologies. Presence of echocardiographic signs of pulmonary arterial hypertension according to 2015 criteria Diagnosis of left heart disease Cumulative smoking > 10 packs.years Infectious pneumonia within 90 days prior to inclusion Acute respiratory illness requiring hospitalization within one year prior to inclusion Pregnant or breastfeeding woman Refusal to participate in the study Refusal to use the data Adults under legal protection (temporary protection measure, curatorship, guardianship)

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 100 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: Diagnostic
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Case group<br>The intervention of the research corresponds to the realization of a thoracic echography. | Other: thoracic echography<br>* The research intervention corresponds to the performance of a thoracic echography to diagnose an incipient pulmonary interstitial syndrome.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Presence of interstitial syndrome on echography | Interstitial syndrome is defined by the total number of B lines is greather than or equal to 10 and/or the average thickness of the pleural line is greater than 3 mm on at least one test site and/or the irregularity score of the pleural line is greater than 16% | At baseline | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Ventilatory mechanics | Measured by spirometry | At Baseline | 
| Ventilatory mechanics | Measured by plethysmography | At Baseline | 
| Ventilatory mechanics | Measured by impulse oscillometry | At Baseline | 
| Ventilatory mechanics | Measured by cardiopulmonary exercise testing | At Baseline | 
| Pulmonary gas exchange | Measured by cardiopulmonary exercise testing | At Baseline | 
| Pulmonary gas exchange | Measured by Diffusing capacity for carbon monoxide | At baseline | 
| Pulmonary gas exchange | Measured by 6-minute walk test | At baseline | 
| Exercise capacity | Measured by cardiopulmonary exercise testing | At baseline | 
| Circulatory response | Measured by cardiopulmonary exercise testing | At baseline | 
| Dyspnoea | Measured by cardiopulmonary exercise testing | At baseline | 
| Dyspnoea | Assessed by Dyspnea12 | At baseline | 
| Dyspnoea | Assessed by Modified Medical Research Council (mMRC) Scale | At baseline | 
| Assessment of Quality of life | Assessed by Cochin 17-item Scleroderma Functional Scale | At baseline | 
| Assessment of Quality of life | Assessed by Saint George's Respiratory Questionnaire (SGRQ) | At baseline | 
| Cough | Evaluation by Leicester Cough Questionnaire - French translation | At baseline |