Pulmonary Langerhans cell histiocytosis (PLCH) belongs to the spectrum of diffuse interstitial cystic pneumonias; it affects young people of both sexes and occurs almost exclusively in tobacco smokers or co-users of tobacco and cannabis. The management of this severe chronic disease is undertaken in specialized centers. A better understanding of the pathogenic mechanisms of the disease has opened up prospects for targeted therapies. However, supporting the abstinence from inhaling noxious materials which determine its prognosis remains the cornerstone of treatment. Patients with PLCH who persist in smoking despite the diagnosis may be very dependent on tobacco, experience significant difficulties in stopping smoking, and must have access to specialist smoking cessation clinics.
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