Diffuse Lung Disease |

Positive PPD: Atypical Presentation of Pulmonary Langerhans Cell Histiocytosis FREE TO VIEW

Omar Abu Naba'a, MBBS; Ali Saeed, MD
Author and Funding Information

University of New Mexico, Albuquerque, NM

Chest. 2013;144(4_MeetingAbstracts):454A. doi:10.1378/chest.1701416
Text Size: A A A
Published online


SESSION TITLE: Interstitial Lung Disease Student/Resident Case Report Posters II

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Pulmonary Langerhans Cell Histiocytosis offers a diagnostic challenge in part because of variable presentation. The average duration of diagnostic delay is close to 6 months. There is no accurate epidemiological data available regarding racial differences. It has rarely been reported in African American females.

CASE PRESENTATION: A 50-year-old woman presented to her PCP for a three months history of cough, exertional dyspnea and 20 pounds weight loss. She had no complaints of fever, chills, rigors, hemoptysis or night sweats. Past history was significant for a 35 pack years of smoking. A chest radiograph showed bilateral nodular infiltrates. She mentioned a remote but a significant exposure to a family member with active tuberculosis. Her PPD test showed 15mm of induration. The patient was cachectic in appearance. There were no palpable lymph nodes. Auscultation of the lungs revealed clear breath sounds. She was recently tested negative for HIV. AFB in Sputum was negative on three different occasions. A high-resolution CT scan showed bilateral pulmonary nodules with different sizes and irregular borders. Pulmonary function testing revealed normal Spirometry and volumes; however the diffusion capacity was moderately reduced. Bronchoscopy with trans-bronchial biopsies was non diagnostic. Patient opted for open lung biopsy. Sections of lung parenchyma showed centrilobular emphysema. Several foci of irregular stellate sclerotic peri-bronchiolar lesions were noted with scattered histiocyte-like cells. The immune histo-chemistry staining for CD1a and S100 came back positive.

DISCUSSION: The management of PCLH is usually individualized. A few may recover spontaneously. All patients should be offered smoking cessation with a 3-6 months radiographic follow up. Some experts recommend prednisone 0.5-1mg/kg/day to patients with symptomatic nodular PLCH. The treatment should be continued then tapered over 6-12 months. Cytotoxic agents have not shown much benefit. Lung transplantation should be considered in patients with aggressive disease and severe pulmonary hypertension. Optimal follow up is based on disease course and patient compliance. A clinic visit should be scheduled every 3-6 months. With the availability of HRCT, It may be appropriate to use for follow up. There are no studies available comparing pulmonary functions testing with HRCT for follow up of these patients.

CONCLUSIONS: The clinical presentation was highly suggestive of milliary tuberculosis. Patient was given the option to start empiric treatment with follow up in 6-8 weeks versus open lung biopsy. Patient chose open lung biopsy revealing a diagnosis of PLCH. Patient was motivated to quit smoking with rapid resolution of cough and exertional dyspnea. Chest CT done after 6 months showed almost complete resolution of the pulmonary nodules.

Reference #1: Sundar, et al. (2003). PLCH: emerging concepts in pathobiology, radiology, and clinical evolution of disease. Chest, 123(5), 1673-1683.

DISCLOSURE: The following authors have nothing to disclose: Omar Abu Naba'a, Ali Saeed

No Product/Research Disclosure Information




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.

Find Similar Articles
CHEST Journal Articles
PubMed Articles
Erdheim-Chester Disease: A Rare Presentation of a Rare Disease. J Investig Med High Impact Case Rep ;4(3):2324709616663233.
Histiocytic proliferations. Semin Diagn Pathol Published online Sep 1, 2016;
  • CHEST Journal
    Print ISSN: 0012-3692
    Online ISSN: 1931-3543