0
Diffuse Lung Disease |

A Case of Pulmonary Langerhans' Cell Histiocytosis Resembling Stage IV Sarcoidosis FREE TO VIEW

Cesar Delgado*, MS; Raquel Aguirre, MD; Silvia Quadrelli, PhD
Author and Funding Information

Hospital Britanico, Buenos Aires, Argentina


Chest. 2012;142(4_MeetingAbstracts):464A. doi:10.1378/chest.1388769
Text Size: A A A
Published online

Abstract

SESSION TYPE: ILD Global Case Report Posters

PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION: Pulmonary Langerhans’ Cell Histioytosis (PLCH) is an uncommon interstitial lung disease with very characteristic radiographic appearance. A case of PLCH with atypical chest HRCT features resembling stage IV sarcoidosis is reported herein.

CASE PRESENTATION: This case report describes a 47 year-old man with dry cough and dyspnea, which had been progressed for over a year. He was a 15 pack-year current smoker with no other significant medical history. Physiologic testing showed a moderate to severe restrictive disease as well as a proportionately reduced diffusing capacity, and a marked decrease in oxygen saturation on the six-minute walk test from 95 to 86%. Chest high-resolution computed tomography (HRCT) revealed irregular enlargement of the bronchovascular bundles, diffuse reticular thickening along with small nodules distributed in a perilynphatic pattern, and upper lobe opacities within traction bronchiectasis. Neither cysts nor costophernic angle sparing was noted. Based on these findings, stage IV sarcoidosis was initially suspected. Open lung biopsy was performed and Pulmonary Langerhans’ Cell Histiocytosis was diagnosed on the basis of several stellated interstitial nodes composed by lymphocytes, eosinophils, and Langerhans’ cells with their characteristic staining for S-100 protein and CD-1a. Further investigation led to the discovery of diabetes insipidus, secondary hypothyroidism, and multiple cystic bone lesions. On diagnosis the patient stopped smoking and was treated with prednisolone for PLCH.

DISCUSSION: PLCH is characterized by clonal proliferation and infiltration of Langerhans’ cells either in isolation or in addition to other organ systems. Chest HRCT is a particularly useful high sensitive diagnostic tool in patients suspected with Pulmonary Langerhans’ Cell Histiocytosis. The typical radiologic finding is the combination of multiple cysts and nodules with a mild to upper zone predominance. The imaging presented in this case is rare in PLCH. Although it has been described a mainly nodular pattern in the early stages of this condition, to our knowledge, this is the first report of a case with severe systemic presentation and a chest HRCT showing a fibrotic reticular nodular distribution without cysts, resembling stage IV sarcoidosis.

CONCLUSIONS: PLCH may present with atypical chest HRCT features, its diagnosis should be considered in young smokers with diffuse lung disease even in the absence of cystic changes.

1) Abbott GF, Rosado-de-Christenson ML, Franks TJ, Frazier AA, Galvin JR. From the archives of the AFIP: Pulmonary Langerhans cell histiocytosis. Radiographics 2004;24:821-41.

2) Canuet M, Kessler R, Jeung MY, et al.Correlation between high-resolution computed tomography findings and lung function in pulmonary Langerhans cell histiocytosis. Respiration 2007; 74:640.

3) Juvet SC, Hwang D, Dwney GP. Rare lung diseases III: pulmonary Langerhans' cell histiocytosis. Can Respir J 2010 May-Jun;17(3):e55-62.

DISCLOSURE: The following authors have nothing to disclose: Cesar Delgado, Raquel Aguirre, Silvia Quadrelli

No Product/Research Disclosure Information

Hospital Britanico, Buenos Aires, Argentina

Figures

Tables

References

NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

CHEST Journal Articles
CHEST Collections
PubMed Articles
  • CHEST Journal
    Print ISSN: 0012-3692
    Online ISSN: 1931-3543