Adult Pulmonary Langerhans Cell Histiocytosis: a National Registry-based Prospective Cohort Study
For patients and families
In plain language
An automatic summary of structured registry data. It is an orientation aid, not a substitute for the official protocol or a physician assessment.
- What is being studied
- The protocol lists: Standard of care.
- Who it may be relevant to
- Registry conditions: Histiocytosis, Histiocytosis, Langerhans-Cell, Histiocytosis Pulmonary. Basic parameters: from 18 years · All.
- What needs checking
- Age, condition and sex are only basic indicators. Prior treatment, laboratory values and other mandatory requirements appear in the eligibility criteria below.
- Where it takes place
- France
- Next step
- Save the trial, show it to the treating physician, and confirm current recruitment with the study center. Costs, documents and travel →
Unsure about the terms? Read our patient guide →
Official title
Enquête épidémiologique Sur la prévalence et le Pronostic de l'Histiocytose Langerhansienne Pulmonaire de l'Adulte en France
Overview
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
- Other Standard of care
Standard of car of pulmonary Langerhans cell histiocytosis (PLCH)
Primary outcome measures
- Overall survival [Time frame: 20 years]
Secondary outcome measures (5)
- CRF [Time frame: 20 years]
- PH [Time frame: 20 years]
- Malignant diseases [Time frame: 20 years]
- Extra-pulmonary involvement in isolated PLCH [Time frame: 20 years]
- Annual prevalence [Time frame: 20 years]
Eligibility criteria
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
\-
Criteria are shown verbatim from the registry (in English). Final eligibility is always assessed by the study center.
Healthy volunteers: No
Study design
- Observational model
- Cohort
Study locations
France · 1 center
- French national reference centre for Histiocytoses — Paris
Publications
- Benattia A, Bugnet E, Walter-Petrich A, de Margerie-Mellon C, Meignin V, Seguin-Givelet A, Lorillon G, Chevret S, Tazi A. Long-term outcomes of adult pulmonary Langerhans cell histiocytosis: a prospective cohort. Eur Respir J. 2022 May 26;59(5):2101017. doi: 10.1183/13993003.01017-2021. Print 2022 May. PMID 34675043
Identifiers
NCT: NCT04665674 · 2014_TAZI