Pneumothorax in Patients with Pulmonary Langerhans Cell Histiocytosis

Radzikowska, E; Błasińska‐Przerwa, Katarzyna; Wiatr, Elżbieta; Bestry, Iwona; R, Langfort; Roszkowski‐Śliż, Kazimierz

doi:10.1007/s00408-018-0155-1

Cited by 14 publications

(16 citation statements)

References 21 publications

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“…The 50% rate of pneumothorax recurrence and the median number of 2 pneumothorax episodes in our study population were somewhat similar to those reported in a previous smaller series of 16 patients [3] and higher than that observed in a more recent larger study [2]. The rate of pneumothorax recurrences after PLCH is significantly lower than what was reported in LAM and BHD syndrome [8–10].…”

Section: Discussionsupporting

confidence: 88%

“…Pneumothorax occurs in approximately 15–20% of PLCH patients [1]. It may be bilateral, recur during the course of the disease, and its management is not standardised [2]. 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].…”

Section: Introductionmentioning

confidence: 99%

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Management and outcomes of pneumothorax in adult patients with Langerhans cell Histiocytosis

Guen

Chevret

Bugnet

et al. 2019

Orphanet J Rare Dis

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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.

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Section: Discussionsupporting

confidence: 88%

Section: Introductionmentioning

confidence: 99%

Management and outcomes of pneumothorax in adult patients with Langerhans cell Histiocytosis

Guen

Chevret

Bugnet

et al. 2019

Orphanet J Rare Dis

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“…Pneumothoraces are less common in children compared to the adult population [ 8 , 18 , 19 ]. R adzikowska et al [ 21 ] found that 32% of their predominantly adult population group had pneumothoraces at initial presentation.…”

Section: Presentationmentioning

confidence: 99%

Paediatric pulmonary Langerhans cell histiocytosis

Barclay

Devaney

Bhatt

2020

Breathe

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Paediatric pulmonary Langerhans cell histiocytosis (pPLCH) is a rare diffuse cystic lung disease. Unlike pulmonary Langerhans cell histiocytosis (LCH) in adults, which is often seen as an isolated condition with smoking being a major risk factor, isolated pPLCH is vanishingly rare in children and it is most often a component of multisystem LCH. Diagnosis should be based on histological and immunophenotypic examination of affected tissue in addition to clinical and radiological features. It should be considered an important differential for diffuse cystic lung disease in paediatric patients. Recent progress in the biological understanding of the disease supports the classification of LCH as an inflammatory myeloid neoplasia. Chemotherapy and specific management of respiratory complications are the mainstays of treatment. The lungs are no longer considered a “risk organ” in LCH as pulmonary involvement is not associated with a worse prognosis than the involvement of other organs. Multidisciplinary treatment approaches are needed.Prognosis can be good but is adversely influenced by multisystem involvement, and complications such as pneumothoraces and respiratory failure can be life threatening. This review aims to give an overview of this condition, with a focus on the diagnosis, monitoring and management of complications such as pneumothoraces and respiratory failure, which can be challenging for the paediatric respiratory specialist.Educational aimsTo give an overview of paediatric pulmonary LCH.To discuss the differential diagnosis of paediatric cystic lung disease.

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“…The diffusing capacity could be impaired by itself or accompanied by restrictive, obstructive or mixed ventilation abnormalities, which was in agreement with previous studies [ 4 , 7 ]. Radzikowska et al presented that patients with pneumothorax had worse FEV, FEV1 and TLC than those without pneumothorax in a cohort involving 90 patients [ 20 ]. We also found that patients with a history of pneumothorax had worse DLCO, FEV1 and FEV1/FVC.…”

Section: Discussionmentioning

confidence: 99%

Clinical presentation and prognostic analysis of adult patients with Langerhans cell histiocytosis with pulmonary involvement

et al. 2020

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Background The study aimed to investigate the clinical features and prognosis factors of adult patients with Langerhans cell histiocytosis (LCH) with pulmonary involvement, especially multisystem (MS) LCH with pulmonary involvement. Methods We retrospectively analyzed the demographic materials, clinical features and treatment outcomes of 119 adult LCH patients with pulmonary involvement at our center from January 1990 to November 2019. Results Among 119 patients, 13 (10.9%) had single-system (SS) LCH, and 106 (89.1%) had MS-LCH with pulmonary involvement. SS-LCH patients had higher smoking rate (84.6% vs 52.8%, P = 0.026) and smoking index (300 vs 200, P = 0.019) than MS-LCH patients. The percentage of respiratory symptoms of SS-LCH patients was higher than MS-LCH patients (84.6% vs 53.8%, P = 0.034). Pulmonary function was impaired in 83.8% of the patients, and DLCO was the parameter most frequently impaired, accounting for 81.1%. The median DLCO was 65.1% predicted. Patients with pneumothorax had significantly worse DLCO (P = 0.022), FEV1 (P = 0.000) and FEV1/FVC (P = 0.000) than those without pneumothorax. During the follow-up, 72.4% of the patients had stable pulmonary function, and 13.8% showed improvements after chemotherapy. The estimated 3-year OS and EFS were 89.7 and 58.3%, respectively. Patients with a baseline FEV1 ≤ 55% predicted had worse OS. A history of pneumothorax indicated worse EFS and cytarabine based therapy predicted better EFS. Conclusions An FEV1 ≤ 55% predicted and a history of pneumothorax at diagnosis indicated a poor prognosis. Cytarabine based regimen may arrest the decline in pulmonary function in LCH patients with pulmonary involvement and improve EFS.

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Pneumothorax in Patients with Pulmonary Langerhans Cell Histiocytosis

Cited by 14 publications

References 21 publications

Management and outcomes of pneumothorax in adult patients with Langerhans cell Histiocytosis

Management and outcomes of pneumothorax in adult patients with Langerhans cell Histiocytosis

Paediatric pulmonary Langerhans cell histiocytosis

Clinical presentation and prognostic analysis of adult patients with Langerhans cell histiocytosis with pulmonary involvement

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