National Study on Long-Term Outcomes and Survival of Adult Pulmonary Langerhans Cell Histiocytosis Patients in France

What this Trial Is About

Researchers are studying adult patients with pulmonary Langerhans cell histiocytosis PLCH to understand their long-term outcomes, especially survival rates. This is the first prospective national study using a large registry of PLCH patients in France, followed over many years to identify risk factors affecting their prognosis. The study is observational and aims to provide valuable information on the disease course in adults. Participants include adults aged 18 years or older who have been newly diagnosed with PLCH. Diagnosis is confirmed either by biopsy or through a combination of clinical signs, specific lung imaging patterns on high-resolution CT scans, and ruling out other diseases. Patients receive standard care for PLCH as part of the study. During the study, researchers will monitor overall survival over 20 years as the main outcome. They will also track additional health data including clinical report forms, pulmonary hypertension, occurrence of malignant diseases, involvement beyond the lungs, and yearly disease prevalence. This long-term follow-up helps to understand the disease progression and patient health over time.

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