Pneumothorax in Pulmonary Langerhans Cell Histiocytosis

doi:10.1378/chest.125.3.1028

Chest

Volume 125, Issue 3, March 2004, Pages 1028-1032

https://doi.org/10.1378/chest.125.3.1028 Get rights and content

Background

Pulmonary Langerhans cell histiocytosis (PLCH) is a smoking-related interstitial lung disease characterized by development of cystic changes that predispose to occurrence of pneumothorax.

Study objectives

To determine the frequency, recurrence rate, and optimal management of pneumothorax associated with PLCH.

Design

Retrospective study.

Setting

Tertiary care, referral medical center.

Patients

One hundred two adults ≥ 18 years old with histologically confirmed PLCH seen at Mayo Clinic Rochester over a 23-year period from 1976 to 1998.

Interventions

None.

Measurements and results

Sixteen of 102 patients (16%) with PLCH had pneumothorax; mean age at the time of diagnosis was 29.4 years (range, 18 to 52 years), and all had smoked cigarettes. There were 37 episodes of pneumothoraces (1 to 5 episodes per patient); 10 patients (63%) had more than one episode. Median age at diagnosis of PLCH was significantly younger in patients with pneumothorax when compared to those without pneumothorax (27 years vs 41.5 years, p < 0.001), but pulmonary function parameters and survival after diagnosis were not significantly different. Rate of recurrent pneumothorax was 58% to the ipsilateral side when the episode was managed by observation or chest tube without pleurodesis, and 0% after surgical management with pleurodesis.

Conclusions

These data support the early use of surgical therapy with pleurodesis in managing patients with PLCH and spontaneous pneumothorax.

Section snippets

Materials and Methods

We identified 102 adults ≥ 18 years old with histologically confirmed PLCH seen at our institution over a 23-year period from January 1, 1976, to December 31, 1998.⁶ Histologic confirmation of PLCH in these patients had been obtained by surgical lung biopsy, transbronchoscopic lung biopsy, or biopsy of an organ other than the lung with findings on a high-resolution CT of the chest that were consistent with the diagnosis. From this cohort of 102 patients, a subset of patients who had

Results

Of 102 adults with histologically confirmed PLCH, 16 patients (16%) had one or more episodes of pneumothorax. There were 12 men and 4 women; median age at the time of PLCH diagnosis was 27 years (range, 18 to 52 years). The age at diagnosis was significantly younger in these patients when compared to the group (86 patients) not having pneumothorax (median age, 27 vs 41.5 years; p < 0.001). Pneumothorax was the initial manifestation of PLCH in 11 of these patients (69%). All initial episodes of

Discussion

A relatively high recurrence rate (58%) was observed in our patients with PLCH when pneumothorax was managed without pleurodesis. Episodes of pneumothorax that were managed with observation only represented cases of small pneumothorax, which likely explains the relatively low rate of recurrence (17%) when compared to the group managed by chest tube drainage (72%). In contrast, there was no recurrent pneumothorax after surgical management that included pleurodesis.

PLCH commonly afflicts

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Citation Excerpt :
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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.
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.
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.
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.
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Chest

Clinical InvestigationsMISCELLANEOUSPneumothorax in Pulmonary Langerhans Cell Histiocytosis

Background

Study objectives

Design

Setting

Patients

Interventions

Measurements and results

Conclusions

Section snippets

Materials and Methods

Results

Discussion

Chest

Chest

Chest

Chest

Pulmonary Langerhans'-cell histiocytosis

N Engl J Med

Adult pulmonary Langerhans' cell histiocytosis

Thorax

Smoking-related interstitial lung diseases: concise review

Eur Respir J

Pulmonary and mediastinal lesions in children with Langerhans cell histiocytosis

Pediatr Radiol

Smoking-related interstitial lung diseases

Curr Opin Pulm Med

Clinical outcomes of pulmonary Langerhans'-cell histiocytosis in adults

N Engl J Med

Pulmonary Langerhans cell granulomatosis (histiocytosis X): a clinicopathologic study of 48 cases

Am J Surg Pathol

Eosinophilic granuloma of lung: clinical aspects of primary pulmonary histiocytosis in the adult

Medicine (Baltimore)

Pulmonary histiocytosis X: pulmonary function and exercise physiology

Am J Respir Crit Care Med

Determinants of survival in pulmonary Langerhans' cell granulomatosis (histiocytosis X)

Eur Respir J

Clinical and radiologic features, lung function and therapeutic results in pulmonary histiocytosis X

Respiration

Non-parametric estimation from incomplete observations

JAMA

Clinical Investigations
MISCELLANEOUS
Pneumothorax in Pulmonary Langerhans Cell Histiocytosis