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Obstructive Lung Diseases |

A Young Sporadic Smoker With Clubbing: An Uncommon Presentation of PLCH

Benjamin Seides, MD; Young Im Lee, MD; Ravindra Rajmane, MD
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NYU School of Medicine, New York, NY


Chest. 2013;144(4_MeetingAbstracts):665A. doi:10.1378/chest.1687534
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Abstract

SESSION TITLE: Interstitial Lung Disease Case Report Posters I

SESSION TYPE: Affiliate Case Report Poster

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

INTRODUCTION: A 32 year old man with a mild smoking history was seen in the office with digital clubbing.

CASE PRESENTATION: A 32 year old man with a mild smoking history was seen in the office with digital clubbing. He reported unlimited exercise tolerance, and denied cardiopulmonary symptoms. PFTs, including lung volume and DLCO, were all within normal limits. CXR, chest CT, and echocardiogram were recommended, but the patient declined further work-up since he was asymptomatic. A year later, the patient was hospitalized for acute appendicitis. Abnormalities seen on basal lung cuts of the abdominal CT prompted chest CT. This revealed diffuse scattered thin-walled cysts, many confluent emphysematous blebs, and reticulonodular opacities. Lymphadenopathy was absent. VATS biopsies of RML/RLL revealed peribronchiolar stellate-shaped fibrotic nodules with traction emphysema, rare eosinophils, focal areas of histiocytic cells, and respiratory bronchiolitis. Immunostaining was S-100 and CD1a positive. These findings were consistent with a diagnosis of pulmonary Langerhans’ cell histiocytosis (PLCH.)

DISCUSSION: PLCH is a rare fibrocystic interstitial lung disease characterized by nodular foci of polyclonal aggregates of Langerhans’ cells (LC) around the bronchovascular bundles of the lung. It affects Caucasians ages 20-40 year old without gender predilection. The pathogenesis of PLCH is unknown. PLCH has a strong association with current or prior tobacco use, but otherwise has no known genetic or environmental causes. PLCH is most frequently diagnosed incidentally, following spontaneous pneumothorax, or in work-up of constitutional symptoms. PLCH causes pneumothorax, hemoptysis, and pulmonary hypertension from venoocclusive disease or arteriopathy. Tobacco cessation is the only effective treatment, and disease regression can occur. Corticosteroids and other immunomodulating drugs have been tried with anecdotal efficacy. Continued smoking, and sometimes despite cessation, results in progressive disease and respiratory failure for which lung transplant is the only option. Disease can recur in transplanted lungs, especially if smoking continues. Our case has numerous unusual features. First, digital clubbing is rarely seen in PLCH. Second, early PLCH often presents with normal spirometry but mildly decreased DLCO. Diminished exercise capacity is often out of proportion to the PFT findings. As disease advances, obstructed PFTs with severely reduced DLCO are seen. However, our patient presented with no cardiopulmonary impairment or PFT abnormalities despite profoundly advanced radiographic disease. Finally, our patient had a modest smoking history, whereas most patients with PLCH present with a more extensive tobacco history.

CONCLUSIONS: Consider PLCH in the young smoker with digital clubbing.

Reference #1: Vassallo R., Ryu JH. Clin Chest Med 25 (2004) 561-571.

Reference #2: Sundar K., et al. Chest 2003; 123:1673-1683.

DISCLOSURE: The following authors have nothing to disclose: Benjamin Seides, Young Im Lee, Ravindra Rajmane

No Product/Research Disclosure Information


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