SESSION TITLE: Interstitial Lung Disease Cases II
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Monday, October 28, 2013 at 04:15 PM - 05:15 PM
INTRODUCTION: Exogenous lipoid pneumonia (ELP) results from inhalation or aspiration of lipid-containing substances into the respiratory tract resulting in tissue inflammation and damage. We describe a patient with chronic severe pulmonary sarcoidosis who developed progressive clinical and radiologic worsening of disease 17 years after the original diagnosis. Lung pathologic findings were consistent with lipoid pneumonia.
CASE PRESENTATION: A 33-year-old man with biopsy-proven pulmonary sarcoidosis diagnosed at age 16 presented with persistent dyspnea and stable severe fibrosis and restriction. He had been maintained on 10 mg of prednisone for many years, resulting in steroid-induced diabetes and obesity; he was treated briefly with methotrexate, which was discontinued due to reported lung toxicity. Upon presentation to our clinic, azathioprine was started as a steroid-sparing agent. Over the span of one year, the patient worsened clinically and radiographically; pulmonary function tests revealed worsening restrictive lung disease. The patient underwent open lung biopsy to assess for alternate or coincident diagnoses. Biopsies of the right upper, middle, and lower lobes revealed lipid-laden macrophages and absent evidence of active infection or sarcoidosis. Careful history subsequently revealed that the patient had applied intranasal petroleum jelly for dry mucous membranes induced by supplemental oxygen therapy for several years.
DISCUSSION: ELP is an uncommon disorder that frequently goes unrecognized for years and may be even less likely to be suspected in patients with other known lung diseases. Our patient’s respiratory symptoms and radiographic findings of architectural distortion, perihilar fibrosis, and diffuse ground glass opacities might easily have been interpreted as progressive severe pulmonary sarcoidosis; however, the subacute progression of symptoms many years after the initial diagnosis led us to investigate alternative diagnoses. In this case, the specific histopathologic evidence of ELP led us to elicit the compatible history and secure the diagnosis. ELP is treated by removal of the offending agent; corticosteroids have been used anecdotally, although some cases progress despite treatment.
CONCLUSIONS: Clinicians should inquire about exposures to lipid-containing substances in patients with interstitial lung disease, of which ELP is an under-recognized cause. The case highlights the importance of considering alternate or coincident diagnoses in patients with acute worsening of chronic interstitial lung disease. Finally, patients on chronic home oxygen supplementation should be counseled against the use of intranasal petroleum jelly to avoid this complication.
Reference #1: Hadda V, et. al. Lipoid Pneumonia: An Overview. Expert Rev Respir Med 2010 Dec;4(6):799-807.
Reference #2: Marchiori E., et al. Exogenous lipoid pneumonia. Clinical and radiological manifestations. Respir Med. 2011 May;105(5):659-66.
DISCLOSURE: The following authors have nothing to disclose: Natoushka Trenard, Keren Bakal, Benjamin Seides, Elizabeth Mulaikal, Caralee Caplan-Shaw
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