Abstract
BackgroundPneumothorax may recur during pulmonary Langerhans cell histiocytosis (PLCH) patients’ follow-up and its management is not standardised. The factors associated with pneumothorax recurrence are unknown.MethodsIn this retrospective study, PLCH patients who experienced a pneumothorax and were followed for at least 6 months after the first episode were eligible. The objectives were to describe the treatment of the initial episode and pneumothorax recurrences during follow-up. We also searched for factors associated with pneumothorax recurrence and evaluated the effect on lung function outcome. Time to recurrence was estimated by the Kaplan Meier method and the cumulative hazard of recurrence handling all recurrent events was estimated. Univariate Cox models and Andersen-Gill counting process were used for statistical analyses.ResultsFourty-three patients (median age 26.5 years [interquartile range (IQR), 22.9–35.4]; 26 men, 39 current smokers) were included and followed for median time of 49 months. Chest tube drainage was the main management of the initial pneumothorax, which resolved in 70% of cases. Pneumothorax recurred in 23 (53%) patients, and overall 96 pneumothoraces were observed during the study period. In the subgroup of patients who experienced pneumothorax recurrence, the median number of episodes per patient was 3 [IQR, 2–4]. All but one recurrence occurred within 2 years after the first episode. Thoracic surgery neither delayed the time of occurrence of the first ipsilateral recurrence nor reduced the overall number of recurrences during the study period, although the rate of recurrence was lower after thoracotomy than following video-assisted thoracic surgery (p = 0.03). At the time of the first pneumothorax, the presence of air trapping on lung function testing was associated with increased risk of recurrence (hazard ratio = 5.08; 95% confidence interval [1.18, 21.8]; p = 0.03). Pneumothorax recurrence did not predict subsequent lung function decline (p = 0.058).ConclusionsOur results show that pneumothorax recurrences occur during an “active” phase of PLCH. In this observational study, the time of occurrence of the first ipsilateral recurrence and the overall number of pneumothorax recurrences were similar after conservative and thoracic surgical treatments. Further studies are needed to determine the best management to reduce the risk of pneumothorax recurrence in PLCH patients.
Highlights
Pneumothorax may recur during pulmonary Langerhans cell histiocytosis (PLCH) patients’ follow-up and its management is not standardised
Study design and subject selection All patients 18 years of age or older with PLCH who were referred to the National Reference Centre for Histiocytoses between November 2003 and December 2015 were eligible for the study, provided they fulfilled the following inclusion criteria: 1) they experienced at least one pneumothorax; 2) information was available on pneumothorax management; 3) they were followed for at least 6 months after the first pneumothorax
The hazard of lung function worsening after pneumothorax recurrence did not reach statistical significance (p = 0.058). In this observational study of 43 PLCH patients experiencing a pneumothorax and followed for a median time of 49.1 months, we found the following salient results: 1) recurrent pneumothorax occurred in approximately half of the patients within 2 years following the initial pneumothorax, and were ipsilateral in three quarters of cases; 2) thoracic surgery did not modify the risk of pneumothorax recurrence; 3) in the univariate analyses, the presence of air trapping on lung function testing was associated with increased risk of pneumothorax recurrence
Summary
Pneumothorax may recur during pulmonary Langerhans cell histiocytosis (PLCH) patients’ follow-up and its management is not standardised. Pulmonary Langerhans cell histiocytosis (PLCH) is a rare diffuse cystic lung disorder that occurs mainly in young smokers of both genders [1]. In adults, it is frequently the only manifestation of the disease, but may be a part of systemic disease [1]. The only one available series of 16 patients reported a high rate of pneumothorax recurrence (58%) after conservative treatment including chest tube drainage, as compared to no recurrence after thoracotomy (TCT) [3]. Whether these results may be extrapolated to that of video-assisted thoracic surgery (VATS) - which is currently the main technical surgery performed for the surgical management of pneumothorax in general [4,5,6] , warrants further evaluation
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