Chest Computed Tomographic Image Screening for Cystic Lung Diseases in Patients with Spontaneous Pneumothorax Is Cost Effective

Abstract

Rationale: Patients without a known history of lung disease presenting with a spontaneous pneumothorax are generally diagnosed as having primary spontaneous pneumothorax. However, occult diffuse cystic lung diseases such as Birt-Hogg-Dubé syndrome (BHD), lymphangioleiomyomatosis (LAM), and pulmonary Langerhans cell histiocytosis (PLCH) can also first present with a spontaneous pneumothorax, and their early identification by high-resolution computed tomographic (HRCT) chest imaging has implications for subsequent management.Objectives: The objective of our study was to evaluate the costeffectiveness of HRCT chest imaging to facilitate early diagnosis of LAM, BHD, and PLCH.Methods: We constructed a Markov state-transition model to assess the cost-effectiveness of screening HRCT to facilitate early diagnosis of diffuse cystic lung diseases in patients presenting with an apparent primary spontaneous pneumothorax. Baseline data for prevalence of BHD, LAM, and PLCH and rates of recurrent pneumothoraces in each of these diseases were derived from the literature. Costs were extracted from 2014 Medicare data. We compared a strategy of HRCT screening followed by pleurodesis in patients with LAM, BHD, or PLCH versus conventional management with no HRCT screening. Measurements and Main Results:In our base case analysis, screening for the presence of BHD, LAM, or PLCH in patients presenting with a spontaneous pneumothorax was cost effective, with a marginal cost-effectiveness ratio of $1,427 per quality-adjusted lifeyear gained. Sensitivity analysis showed that screening HRCT remained cost effective for diffuse cystic lung diseases prevalence as low as 0.01%. Conclusions:HRCT image screening for BHD, LAM, and PLCH in patients with apparent primary spontaneous pneumothorax is cost effective. Clinicians should consider performing a screening HRCT in patients presenting with apparent primary spontaneous pneumothorax.

Spontaneous pneumothorax in diffuse cystic lung diseases

“…Current guidelines do not recommend screening CT for first time pneumothoraces (10). However, in a recent study, performing a screening HRCT to facilitate early diagnosis of LAM, BHD, or PLCH followed by pleurodesis was found to be cost-effective with a marginal cost-effectiveness ratio of $1,427 per quality-adjust life-year (QALY) gained, which is substantially lower than the commonly accepted threshold of $50,000/QALY (95). Based on these results, we recommend that all patients with an apparent primary spontaneous pneumothorax be screened with HRCT for the presence of underlying DCLDs.…”

“…Recent data suggests that BHD, LAM, and PLCH likely cause approximately 10% of apparent primary spontaneous pneumothoraces in the general population (61, 62, 94, 95). Current guidelines do not recommend screening CT for first time pneumothoraces (10).…”

Pulmonary Langerhans' Cell Histiocytosis in Adults

“…Pleural lesions due to past pneumothorax are frequently observed. Moreover, enlargement of the hilar or mediastinal lymph nodes may occur [3,[7][8][9][10][11][12][24][25][26].…”

“…Computed tomography, in particular high-resolution computed tomography, is important in diagnosis of histiocystic lesions, especially in the case of the lungs [26]. The most often noted lesions are centrilobular nodules, frequently forming 'tree-in-bud' appearance, nodules with or without a lacuna, initially thick-walled cysts of various shape, which may be isolated or confluent with the appearance of "clover leaves".…”

“…Moreover, this group often includes young men who smoke relatively less cigarettes and whose lung function is more affected by the disease. Taking into consideration the above data and clinical and financial effectiveness of HRCT in identifying PLCH patients, recommendation of HRCT in the case of the individual with spontaneous pneumothorax seems to be justified [24][25][26].…”