National Lymphangioleiomyomatosis Registry, France

Recruiting

• Conditions: Lymphangioleiomyomatosis

• Registration Number: NCT01484236
• Lead Sponsor: Vincent COTTIN

Brief Summary

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

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

Study Timeline

• Study First Submitted: 2011-11-30
• Study First Submitted QC: 2011-12-01
• Study First Posted: 2011-12-02
• Study Start: 2012-01
• Status Verified: 2025-03
• Last Update Submitted: 2025-03-11
• Last Update Posted: 2025-03-13
• Primary Completion: 2025-12
• Study Completion: 2026-12

Recruitment & Eligibility

• Status: RECRUITING
• Sex: All
• Target Recruitment: 200

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

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Number of cases	4 years	Determine incidence and prevalence of lymphangioleiomyomatosis.

Secondary Outcome Measures

Name	Time	Method
localisation of cases	4 years	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)
Progression-free survival	4 years	Determine applicability of diagnostic criteria proposed in 2009

Trial Locations

Locations (1)

Hôpital Louis Pradel; 🇫🇷 Lyon, France