In Vivo Endomicroscopic Description of Pulmonary Microcirculation in Systemic Sclerosis and Emphysema Patients

Study Overview
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Brief Summary
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Systemic sclerosis (SSc) is a generalized disorder of connective tissue, arterioles and microvessels, characterized by the occurrence of fibrosis and vascular obliteration phenomena. The alterations in lung microvessels are found in pulmonary involvement of scleroderma, which are the most serious complications of the disease. In pulmonary emphysema, there are also changes in pulmonary microvasculature, which are involved in the onset and development of the disease. The confocal endomicroscopy is an endoscopic technique which can be performed during a bronchoscopy. This technique makes it possible to observe in real time the most distal pulmonary elements at the microscopic scale. After injection of fluorescein, then the technique of observing the pulmonary microvasculature, in vivo and in situ. The characterization of microvascular lesions in these two pathologies could improve understanding of their mechanisms and ultimately improve the early management of patients.

Official Title
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In Vivo Endomicroscopic Description of Pulmonary Microcirculation in Systemic Sclerosis and Emphysema Patients

Conditions
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Pulmonary Disease, Pulmonary Emphysema

Intervention / Treatment
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* Device: Bronchoscopy with in vivo confocal endomicroscopy


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patients with scleroderma with diffuse interstitial pneumonia, indicating a bronchoscopy. Patients with pulmonary emphysema indication for bronchoscopy. Age greater than 18 years. Having an affiliation to social security. Signed informed consent. Exclusion Criteria: Severe chronic respiratory insufficiency against-indicating the completion of a bronchoscopy Hypercapnia defined by a PaCO2 ≥ 6 kPa Disorders of hemostasis against-indicating performing a bronchoscopy Anticoagulant therapy can not be interrupted time of completion of the examination A history of pneumonectomy or contralateral exploration in a nonfunctional lung Contraindication to the injection of fluorescein (including treatment with beta-blocker eye drops or po) or one of its excipients History of an injection poorly tolerated fluorescein A history of food allergy or drug known Myocardial infarction <1 month Unstable angina Pregnant or lactating woman, premenopausal women without adequate contraception Person under guardianship Patient participating in another trial / participating in another trial within 4 weeks

Ages Eligible for Study
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Minimum Age: 18 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
Allocation: Non-Randomized
Intervention Model: Parallel Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: patients with a suspicion of emphysema<br>A bronchoscopy with in vivo confocal endomicroscopy is assessed for patient with a suspicion of emphysema | Device: Bronchoscopy with in vivo confocal endomicroscopy<br>* A bronchoscopy with in vivo confocal endomicroscopy is done for patients with a suspicion of emphysema or for patients with a suspicion of scleroderma<br>|
| Experimental: patients with a suspicion of scleroderma<br>A bronchoscopy with in vivo confocal endomicroscopy is assessed for patient with a suspicion of sclerodermia | Device: Bronchoscopy with in vivo confocal endomicroscopy<br>* A bronchoscopy with in vivo confocal endomicroscopy is done for patients with a suspicion of emphysema or for patients with a suspicion of scleroderma<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Measurement of extra-alveolar diameter of capillaries of patient with pulmonary emphysema or pulmonary scleroderma | Extra-alveolar diameter of capillaries is assessed using in vivo confocal endomicroscopy | Day 1 | 
| Measurement of intercapillary distance of patient with pulmonary emphysema or pulmonary scleroderma | Intercapillary distance is assessed using in vivo confocal endomicroscopy | Day 1 | 
| Measurement of length of the capillary portions of patient with pulmonary emphysema or pulmonary scleroderma | Length of the capillary is assessed using in vivo confocal endomicroscopy | Day 1 | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Measurement of alveolar diameter entries of patient with pulmonary emphysema or pulmonary scleroderma | Alveolar diameter entries is assessed using in vivo confocal endomicroscopy | Day 1 | 
| Measurement of axial thickness of elastic fibers of patient with pulmonary emphysema or pulmonary scleroderma | Axial thickness of elastic fibers is assessed using in vivo confocal endomicroscopy | Day 1 |