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

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

Status

Recruiting

Phases

Unknown

Study type

Observational

Source

ClinicalTrials.gov

Registry ID

NCT04665674

Enrollment

500

Registered

2020-12-11

Start date

2004-01-01

Completion date

2035-12-31

Last updated

2020-12-17

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

Histiocytosis, Histiocytosis, Langerhans-Cell, Histiocytosis Pulmonary

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.

Interventions

OTHERStandard of care

Standard of car of pulmonary Langerhans cell histiocytosis (PLCH)

Study design

Observational model

COHORT

Time perspective

PROSPECTIVE

Eligibility

Sex/Gender

ALL

Age

18 Years to No maximum

Healthy volunteers

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

Design outcomes

Primary

Measure	Time frame	Description
Overall survival	20 years	Overall survival defined as the time from inclusion to death from any cause.

Secondary

Measure	Time frame	Description
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

Countries

France

Contacts

Primary ContactAbdellatif TAZI, MD PhD

abdellatif.tazi@aphp.fr142499198

Backup Contactmatthieu resche-rigon, MD PhD

matthieu.resche-rigon@u-paris.fr142499742

Outcome results

None listed

Source: ClinicalTrials.gov · Data processed: Feb 4, 2026