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

Recruiting

• Conditions: Histiocytosis, Langerhans-Cell; Histiocytosis Pulmonary; Histiocytosis
• Interventions: Other: Standard of care

• Registration Number: NCT04665674
• Lead Sponsor: Assistance Publique - Hôpitaux de Paris

Brief 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.

Detailed Description

Not available

Study Timeline

• Study Start: 2004-01-01
• Status Verified: 2020-11
• Study First Submitted: 2020-11-30
• Study First Submitted QC: 2020-12-07
• Study First Posted: 2020-12-11
• Last Update Submitted: 2020-12-15
• Last Update Posted: 2020-12-17
• Primary Completion: 2034-12-31
• Study Completion: 2035-12-31

Recruitment & Eligibility

• Status: RECRUITING
• Sex: All
• Target Recruitment: 500

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

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
Pulmonary Langerhans cell histiocytosis (PLCH)	Standard of care	All patients with newly diagnosed PLCH at adulthood (i.e. 18 years of age or older) referred to the French national reference centre for Histiocytoses

Primary Outcome Measures

Name	Time	Method
Overall survival	20 years	Overall survival defined as the time from inclusion to death from any cause.

Secondary Outcome Measures

Name	Time	Method
CRF	20 years	Cumulative incidence of chronic respiratory failure (CRF) from inclusion
PH	20 years	Cumulative incidence of pulmonary hypertension (PH) from inclusion
Malignant diseases	20 years	Cumulative incidence of Malignant diseases from inclusion
Extra-pulmonary involvement in isolated PLCH	20 years	Cumulative incidence of extra pulmonary localisations from inclusion
Annual prevalence	20 years	Number of people with the disease at any time during a year

Trial Locations

Locations (1)

French national reference centre for Histiocytoses; 🇫🇷 Paris, France