This protocol covers isolated lung involvement by Langerhans cell histiocytosis in an adult with active or prior inhalation exposure — a predominantly smoking-related presentation managed with a distinct first-line approach.
Single-system pulmonary LCH with isolated lung involvement in an adult smoker. Tobacco, marijuana, vaping, and other inhaled substances are established drivers of this presentation, and disease activity is directly linked to continued exposure.
First-line management centres on complete cessation of all inhaled substances; in select patients with obstructive symptoms, an additional inhaled therapy may be considered. The full evidence-based regimen is available via the button below.
Complete remission of pulmonary LCH, evaluated with pulmonary function tests and high-resolution CT of the chest at 3–6 months.
DOI: 10.1182/blood.2021014343
Single-system pulmonary | Isolated lung involvement (predominantly smoking related).
Cessation of smoking, vaping, inhalation of marijuana or other substances is recommended as first-line therapy for single-system PLCH.
In all cases, smoking cessation (all forms, including marijuana and vaping) is mandatory, which remains difficult to achieve in practice.
It is often the only required intervention that results in complete remission.
Combined inhaled corticosteroid and long-acting b2-agonist therapy may provide benefit to patients with wheezing and moderate obstructive lung disease.
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