National Lymphangioleiomyomatosis Registry, France

Study Overview
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Brief Summary
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RE-LAM-CE is a registry of lymphangioleiomyomatosis cases in France. its aim is to determine the incidence and prevalence of lymphangioleiomyomatosis in France, including demographic information and information concerning the timing and modalities of diagnosis. Based on the registry, we will set up a prospective cohort of patients. The database will include detailed medical information, particularly regarding progression of the respiratory function.

Detailed Description
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Lymphangioleiomyomatosis is a rare pulmonary disease occuring preferentially in women. Few data are available regarding the epidemiology of this disease, the frequency of the various forms with mild to moderate or severe disease, and the rapid or slow progression. Several sources of information will be used in order to increase completeness. The registry will also allow to estimate the proportion of patients with lymphangioleiomyomatosis who are managed by the Reference Center and Competence Centers for rare lung diseases in France. Data will be collected by a research assistant, and the study will be coordinated by the Pole IMER - Department of Public health and Epidemiology of HCL (Lyon Hospitals). This study will provide a better knowledge on the natural course of the disease and the variability in severity and progression of lymphangioleiomyomatosis, and the applicability of diagnostic criteria proposed in 2009. This study will provide the first detailed epidemiological information on Lymphangioleiomyomatosis, will assess the management of this rare disease within the French National Plan for rare diseases 2005 - 2009, and will provide valuable medical information prior to the establishment of clinical trials

Official Title
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National Lymphangioleiomyomatosis Registry, France

Conditions
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Lymphangioleiomyomatosis

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patients with sporadic lymphangioleiomyomatosis or associated with Tuberous sclerosis. patients diagnosed or hospitalized or seen in consultation since 01/01/2008 Exclusion Criteria: None

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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What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Number of cases | Determine incidence and prevalence of lymphangioleiomyomatosis. | 4 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| localisation of cases | Estimate the proportion of patients with lymphangioleiomyomatosis who are managed by the Reference Center and Centers of excellence for rare lung diseases in France (coverage rate of centers) | 4 years | 
| Progression-free survival | Determine the frequency of the various forms with mild to moderate or severe, and rapid or slow evolution. | 4 years | 
| Progression-free survival | Determine applicability of diagnostic criteria proposed in 2009 | 4 years | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Lymphangioleiomyomatosis