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

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.

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:

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