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Lung cancer in patients with CPFE syndrome represents a specific entity with a poor prognosis, that further represents the most characteristic and severe model of tobacco-related disease.

To the Editors:Pulmonary Langerhans' cell histiocytosis (LCH)/histiocytosis X is a rare smoking-related interstitial lung disease that predominantly affects adults aged 20-40 yrs. It is commonly associated with irreversible airflow obstruction and highly variable clinical outcomes. The disease may regress spontaneously or following smoking cessation; however, in 20-30% of cases it progresses to chronic respiratory insufficiency. Despite encouraging results in case series and reports [1], no established efficacy on disease outcomes has been demonstrated with corticosteroids, vinblastine or cladribine.Although rare in patients with early disease, severe precapillary pulmonary hypertension (PH; group 5 in the clinical classification of PH) [2] is frequent in patients with advanced pulmonary LCH. Precapillary PH was present in all 21 consecutive pulmonary LCH patients [3], and in 92% of 39 patients referred for lung transplantation [4] in two series. Lung transplantation is considered the therapy of choice for end-stage pulmonary LCH and chronic respiratory insufficiency, especially among patients who exhibit severe PH [4]. Whether therapy for PH may affect long-term outcomes of disease and impact timing of transplantation is currently unknown. Here, we report the long-term improvement of LCH-associated PH in a patient treated using the dual endothelin receptor antagonist bosentan, obviating the need for lung transplantation.

Severe Kco reduction is frequently related to pulmonary hypertension, especially when associated with emphysema and/or interstitial lung disease. Systematic echocardiography is thus warranted in any patient with severe Kco diminution.

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