A UNIQUE PRESENTATION OF EOSINOPHILIC GRANULOMA IN THE LUNG

Abstract

SESSION TITLE: Diffuse Lung Disease 1 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 pm - 02:15 pm INTRODUCTION: Langerhans Cell Histiocytosis is characterized by granuloma formation in various organs. Pulmonary Langerhans Cell Histiocytosis (PLCH) is a more localized, rare disease. We present a case of a rapidly progressive interstitial lung disease. CASE PRESENTATION: A 58-year-old female, active smoker, with a history of chronic obstructive pulmonary disease (COPD) was referred to the pulmonology clinic for an incidental finding of lung nodules on chest imaging. She presented with upper respiratory tract symptoms to an urgent care center where a chest X-ray was done and showed pulmonary nodules. A CT of the chest then revealed multiple bilateral pulmonary nodules in the upper and middle lung zones, with the largest nodule measuring 12 x 10 mm without associated lymphadenopathy. Of note, these nodules were absent on CT imaging three months prior. She denied dyspnea, cough, fever, night sweats, weight loss, recent travel, exposure to tuberculosis, birds or chemicals. Vital signs and physical exam were unremarkable. Antinuclear antibodies, Cryptococcal antigen and Fungitell assay were negative. Position Emission Tomography was done and showed mild tracer uptake in the largest nodule (left lower lobe). An Interventional Radiology guided biopsy was done and histopathology showed bronchoalveolar tissue infiltrated with Langerhans cells and eosinophils. These Langerhans cells were highlighted by immunohistochemical stains of CD1a and S100, consistent with histiocytosis. She was advised to stop smoking with close follow up. DISCUSSION: Pulmonary Langerhans Cell Histiocytosis (PLCH) is rare and at least 90% of PLCH patients are active smokers [1].PLCH may present with pulmonary symptoms such as dyspnea or non-productive cough; extra-pulmonary manifestations such as bone pain, rash, diabetes insipidus, lymphadenopathy, and weight loss; or patients can be asymptomatic as seen in this case [3]. Diagnosis is by findings on high-resolution CT imaging, and biopsy proven histology with positive immunohistochemical staining for CD1a and S100.PLCH typically affects upper and middle lung zones with sparing of the costophrenic angles and the progression is insidious. However, it was interesting that this patient’s nodules involved bilateral lower lobes and progressed rapidly in size and number over a short interval of three-months.Treatment is individualized as little is known about the pathophysiology of the disease. Data on other treatment modalities is controversial; however smoking cessation has been shown to improve outcomes [2]. CONCLUSIONS: PLCH in elderly smokers may be mistaken for metastatic lung disease. Definitive diagnosis is by histopathology. Therefore, it is important to keep PLCH in the differential diagnosis in patients with diffuse pulmonary nodules, especially because it can be reversed with smoking cessation. Also, missing the diagnosis and delays in treatment can lead to lung fibrosis. Reference #1: Elia D, Torre O, Cassandro R, Caminati A, Harari S. Pulmonary Langerhans Cell Histiocytosis: A comprehensive analysis of 40 patients and literature review. Eur J Intern Med. 2015;26(5):351-356. Reference #2: Wolters PJ, Elicker BM. Subacute onset of Pulmonary Langerhans Cell Histiocytosis with resolution after smoking cessation. Am J Respir Crit Care Med. 2014;190(11):e64. Reference #3: Juvet SC, Hwang D, Downey GP. Rare lung diseases III: Pulmonary Langerhans Cell Histiocytosis. Can Respir J. 2010;17(3):e55-62. DISCLOSURES: No relevant relationships by Gunjan Gupta, source=Web Response no disclosure on file for Lirim Krveshi; No relevant relationships by QUOC NGUYEN, source=Web Response No relevant relationships by Rahila Ogunnaike, source=Web Response No relevant relationships by Aashutosh Ramakar, source=Web Response no disclosure on file for Sakshi Sethi