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Endogenous Lipoid Pneumonia in Pulmonary Alveolar Proteinosis FREE TO VIEW

Adam Kaiser, MD; Margaret Grimes, MD; Kristin Miller, MD
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Virginia Commonwealth University, Richmond, VA

Chest. 2014;146(4_MeetingAbstracts):334A. doi:10.1378/chest.1988334
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SESSION TITLE: Miscellaneous Case Report Posters II

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Lipoid pneumonia (LP) is the filling of the airspace with lipid material with subsequent inflammation. We present a case of endogenous lipoid pneumonia (ELP) occurring in a man with pulmonary alveolar proteinosis (PAP).

CASE PRESENTATION: A 46 year-old man with poorly controlled undifferentiated connective tissue disease (UCTD) despite therapy with methotrexate and biologic agents presented with hypoxic respiratory failure. Initial symptoms were subacute progressive dyspnea and nonproductive cough. CT revealed crazy-paving and nodular infiltrates. He was treated with broad-spectrum antibiotics without improvement. Differential diagnosis included infection, diffuse alveolar hemorrhage, medication-induced lung injury or due to UCTD. Bronchoalveolar lavage (BAL) revealed PAP. He had a two-month hospitalization complicated by respiratory failure requiring mechanical ventilation and infections including Mycobacterium Avium Complex and Aspergillus. Due to lack of improvement, open lung biopsy was pursued and revealed ELP, interstitial fibrosis, and evidence of resolving PAP. His hypoxia improved with corticosteroids and supportive care. Whole lung lavage was not performed due to improvements in imaging and status. At follow-up, he no longer requires supplemental oxygen.

DISCUSSION: LP can be classified as either exogenous or endogenous. ELP occurs after alveolar pneumocyte damage with release of lipid material into the airspace. Unregulated uptake by alveolar macrophages incites inflammation resulting in pneumonitis and potentially fibrosis. ELP can occur in post-obstructive pneumonitis, but has also been associated with other diseases: PAP, mycobacterial and fungal infections, lipid storage disorders, fat embolism, granulomatosis with polyangiitis, and other connective tissue disorders. Presentation ranges from no symptoms to fulminant respiratory failure. Imaging includes focal consolidation, ground glass opacities, crazy-paving, and/or pulmonary nodules. Diagnosis can be made with BAL demonstrating foamy, lipid-laden macrophages or by lung biopsy. Treatment includes relieving infectious or obstructive process, whole lung lavage, and/or corticosteroids.

CONCLUSIONS: LP is a rare, under-diagnosed disease due its non-specific presentation. Biopsy can help establish a diagnosis. It can be associated with PAP. Clinicians should be aware of this as it is unlikely that patients will improve with antibiotics alone.

Reference #1: Harris K, et al. Lipoid pneumonia: a challenging diagnosis. Heart Lung. 2011 Nov-Dec;40(6):580-4.

DISCLOSURE: The following authors have nothing to disclose: Adam Kaiser, Margaret Grimes, Kristin Miller

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