Diffuse Lung Disease |

Beyond Cavities and Emphysema FREE TO VIEW

Akshu Balwan, MD; Thomas Van der Kloot, MD; Jennifer Palminteri, MD
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Maine Medical Center, Portland, ME

Chest. 2015;148(4_MeetingAbstracts):378A. doi:10.1378/chest.2261087
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SESSION TITLE: Diffuse Lung Disease Case Report Posters

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Pulmonary air-filled radiolucencies are classified into cavities, cysts, emphysema or honeycombing based on radiographic and pathological characteristics. While cavities and bullous emphysema are not uncommon, cystic lung diseases are rare and merit discussion. We report a case of a rare cystic lung disease.

CASE PRESENTATION: A65 year-old female was referred for evaluation of an abnormal CXR after presenting with cough. Medical history was notable for hypothyroidism, and monoclonal gammopathy. She had no infectious or constitutional symptoms. Her only recent travel was to Central America. She was a never smoker with no toxic exposure. She reported no relevant family history. Physical examination was unremarkable and basic laboratory evaluation was unrevealing. Her CXR showed lower lung zone predominant large air filled lucencies without air fluid levels. Chest CT showed diffuse cysts and a right lower lobe(RLL) focal mass-like consolidation(Figure 1). Pulmonary function testing showed moderate reduction in DLCO. Serological testing returned positive for SS-A and SS-B antibody. Biopsy of the RLL consolidation showed distention of interstitial spaces with dense mononuclear infiltrate of plasma cell, lymphocytes and histiocytes consistent with lymphocytic interstitial pneumonia (LIP).

DISCUSSION: Lymphocytic interstitial pneumonia(LIP) is rare disease seen mostly in women in the fourth to seventh decade of life. Sjogren's disease is strongly associated with LIP.1 Other associations include dysproteinemia, HIV and HTLV infection, celiac sprue and bone marrow transplantation. Other causes of cystic lung disease include Pulmonary Langerhans cells histiocytosis, lymphangioleiomyomatosis, Birt-Hogg-Dube syndrome, and rarely; desquamative interstitial pneumonia and cystic metastases.2 Almost all other cystic diseases generally present before the age of 50 and have variable association with smoking. Lymphoma can develop in about 5% of patient with LIP.2 This prompted biopsy in our patient (Figure 2). After diagnosis, she refused treatment with steroids.

CONCLUSIONS: Cystic lung diseases are rare and should be considered in the differential diagnosis when there is radiographic evidence of radiolucencies. Rare transformation to lymphoma can be seen in LIP and should warrant further investigation.

Reference #1: Strilman C. etal. Pulmonary manifestations of Sjogren’s syndrome. Chest 1976; 70:354-61

Reference #2: Cosgrove GP. etal. Challenges in pulmonary fibrosis-Cystic lung disease . Thorax 2007; 62:820-829

DISCLOSURE: The following authors have nothing to disclose: Akshu Balwan, Thomas Van der Kloot, Jennifer Palminteri

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