Spontaneous pneumothorax and air travel in Pulmonary Langerhans cell histiocytosis: A patient survey

Abhishek Singla, Elizabeth J Kopras, Nishant Gupta, Abhishek Singla, Elizabeth J Kopras, Nishant Gupta

Abstract

Background: The optimal approach for management of spontaneous pneumothoraces (SPs) and the safety of air travel in patients with pulmonary Langerhans cell histiocytosis (PLCH) are not well established.

Methods: Patients with PLCH were recruited from the Rare Lung Diseases Clinic Network and the Histiocytosis Association, and surveyed about disease manifestations and safety of air travel.

Results: A total of 94 patients completed the survey. Median age at diagnosis of PLCH was 40 years (range: 15-67 years). Average interval between symptom onset and diagnosis was 2.9 years (range: -4 to 31 years). Twenty-two patients (23%) had at least one SP, of which 14 (64%) had at least one additional SP that showed either an ipsilateral recurrence (10 patients; 45%) or a contralateral recurrence (8 patients; 36%). Mean age at the time of first SP was 29 years. SP was the presenting manifestation that led to the diagnosis of PLCH in 19% of patients, typically after the second episode. Surgical pleurodesis reduced the recurrence rate of SP by half in comparison with conservative management (29% vs. 65%, p = 0.025). Two patients experienced an episode of SP during air travel, consistent with an air travel-related pneumothorax rate of 2.4% per patient and 0.27% per flight.

Conclusions: SP is a common manifestation of PLCH, can be seen in approximately one-fourth of the patients, and has a high recurrence risk. Surgical pleurodesis leads to a substantial reduction in the SP recurrence risk. The risk of an air travel-related SP in patients with PLCH is about 2-3 per thousand flights. TRIAL REGISTRY CLINICALTRIALS.GOV: NCT03052101.

Keywords: Air travel; PLCH; Pleurodesis; Pneumothorax.

Conflict of interest statement

Conflict of Interest: The authors report no conflict of interest.

Copyright © 2019 The Japanese Respiratory Society. All rights reserved.

Figures

Figure 1:
Figure 1:
Incidence and recurrence of ipsilateral and contralateral pneumothoraces in PLCH. SP = Spontaneous pneumothorax.
Figure 2:
Figure 2:
Histogram depicting the relationship between age (in years) at first pneumothorax and second pneumothorax and age at diagnosis of PLCH. Most patients developed a pneumothorax in the 2nd and 3rd decade, usually before the diagnosis of PLCH was established.
Figure 3:
Figure 3:
Effectiveness of the treatment of pneumothorax in patients with PLCH. Each hemithorax was considered separately. The highest recurrence rates of pneumothorax occurred with conservative therapy, but surgical and chemical pleurodesis procedures also frequently failed. After the first recurrence episode, all conservatively managed patients experienced a recurrent pneumothorax. * Includes observation, simple aspiration, and tube thoracostomy without pleurodesis # Intervation and/or recurrence details not available for 1 patient with first pneumothorax and 1 patient with second pneumothorax

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