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.
Study Type
OBSERVATIONAL
Enrollment
500
Standard of car of pulmonary Langerhans cell histiocytosis (PLCH)
French national reference centre for Histiocytoses
Paris, France
RECRUITINGOverall survival
Overall survival defined as the time from inclusion to death from any cause.
Time frame: 20 years
CRF
Cumulative incidence of chronic respiratory failure (CRF) from inclusion
Time frame: 20 years
PH
Cumulative incidence of pulmonary hypertension (PH) from inclusion
Time frame: 20 years
Malignant diseases
Cumulative incidence of Malignant diseases from inclusion
Time frame: 20 years
Extra-pulmonary involvement in isolated PLCH
Cumulative incidence of extra pulmonary localisations from inclusion
Time frame: 20 years
Annual prevalence
Number of people with the disease at any time during a year
Time frame: 20 years
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