Adult Pulmonary Langerhans Cell Histiocytosis: a National Registry-based Prospective Cohort Study

Histiocytosis Clinical Trial

Official title:

Enquête épidémiologique Sur la prévalence et le Pronostic de l'Histiocytose Langerhansienne Pulmonaire de l'Adulte en France

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT04665674
• Other study ID #: 2014_TAZI
• Status: Recruiting
• Start date: January 1, 2004
• Est. completion date: December 31, 2035

Study information

• Verified date: November 2020
• Source: Assistance Publique - Hôpitaux de Paris
• Contact: Abdellatif TAZI, MD PhD
• Phone: 142499198
• Email: abdellatif.tazi[@]aphp.fr
• Is FDA regulated: No
• Study type: Observational [Patient Registry]

Clinical Trial Summary

The long-term outcomes of adult patients with pulmonary Langerhans cell histiocytosis (PLCH), particularly survival, is largely unknown. This is the first prospective study in the field evaluating the long-term outcomes of PLCH patients. This french countrywide registry-based study included a large cohort of PLCH patients followed for a sufficiently long period to address risk factors of long-term outcomes of PLCH patients.

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 500
• Est. completion date: December 31, 2035
• Est. primary completion date: December 31, 2034
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years and older

Eligibility

Inclusion Criteria:

• Age >= 18 years

• Diagnosis of PLCH was either histologically confirmed on a biopsy of an involved tissue, or based on the combination of the following criteria: 1) an appropriate clinical picture; 2) a typical nodulo-cystic pattern on lung high-resolution computed tomography (HRCT) and 3) exclusion of alternative diagnoses

Exclusion Criteria:

-

Related Conditions & MeSH terms

• Histiocytosis
• Histiocytosis Pulmonary
• Histiocytosis, Langerhans-Cell

Intervention

Other:
• Standard of care
Standard of car of pulmonary Langerhans cell histiocytosis (PLCH)

Locations

Country	Name	City	State
France	French national reference centre for Histiocytoses	Paris

Sponsors (1)

Lead Sponsor	Collaborator
Assistance Publique - Hôpitaux de Paris

Country where clinical trial is conducted

France, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Overall survival	Overall survival defined as the time from inclusion to death from any cause.	20 years
Secondary	CRF	Cumulative incidence of chronic respiratory failure (CRF) from inclusion	20 years
Secondary	PH	Cumulative incidence of pulmonary hypertension (PH) from inclusion	20 years
Secondary	Malignant diseases	Cumulative incidence of Malignant diseases from inclusion	20 years
Secondary	Extra-pulmonary involvement in isolated PLCH	Cumulative incidence of extra pulmonary localisations from inclusion	20 years
Secondary	Annual prevalence	Number of people with the disease at any time during a year	20 years