Correct!
5. Smoking cessation
No randomized trials of therapy for adult PLCH have yet been reported. Data regarding the effectiveness of interventions for PLCH are derived from observational studies, case reports and expert opinion. The majority of PLCH patients are current or former smokers, it is likely that smoking plays a role in the pathogenesis of the disease. Case reports suggest that smoking cessation may lead to marked improvement or resolution of the disease. However, relapse or worsening despite smoking cessation have also been described. Nevertheless, because of the high rate of PLCH-associated pulmonary malignancy, the established relationship between smoking and airflow obstruction and many other health benefits, smoking cessation is the most important step in the management of PLCH and would be most appropriate for this patient with mild disease.
Approximately 50% of patients have favorable outcomes (1). 10-20% of patients have recurrent pneumothorax or progressive respiratory failure with cor pulmonale. 30-40% of patients have persistent symptoms with conversion of the nodules into a more cystic pattern. Poor prognostic indicators include (1):
Corticosteroids are frequently used in the management of PLCH despite limited evidence of benefit (3). No trials of treatment with mycophenolate have been reported. Similarly, cytotoxic drugs such as vinblastine, cyclophosphamide, methotrexate, 2-chlorodeoxyadenosine (cladribine) and etoposide have been used especially for multisystem disease. Like the corticosteroids there is limited evidence of benefit. Pneumothorax due to PLCH is frequent. Pleurodesis is generally recommended when pneumothorax occurs because the recurrence rate is high. Lung transplantation for PLCH is performed when severe respiratory failure develops. PLCH is known to recur in the allograft following lung transplantation. None of these therapies seem appropriate for this patient with mild disease.
Our patient is currently attempting to quit smoking. Recidivism to smoking appears to be quite high in PLCH patients.
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