Table 8 Therapeutic recommendations in pLCH
Recommendation | Grade |
|---|---|
First step is smoking cessation in all patients | C2 |
Watchful waiting in a- or minor symptomatic patients | C2 |
Systemic steroid therapy in symptomatic patients | C2 |
Chemotherapy (e.g. 2-CDA) in progressive disease | C2 |
Consider lung transplantation in case of severe respiratory failure or major pulmonary hypertension | C2 |