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Diffuse Lung Disease |

Hypersensitivity Pneumonitis Mimicking Lymphangioleiomyomatosis

Anita Rajagopal, MD; Michael Shapiro, MD
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Department of Internal Medicine, St Vincent Medical Center, Indianapolis, IN


Chest. 2014;145(3_MeetingAbstracts):236A. doi:10.1378/chest.1775447
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Abstract

SESSION TITLE: Interstitial Lung Disease Cases

SESSION TYPE: Case Reports

PRESENTED ON: Saturday, March 22, 2014 at 09:00 AM - 10:00 AM

INTRODUCTION: Hypersensitivity pneumonitis (HP) is characterized by diffuse inflammation of lung parenchyma. Symptoms include cough, fever, and shortness of breath. Chest imaging often shows micronodules and fibrosis that can be difficult to distinguish from idiopathic pulmonary fibrosis. We present a case of HP presenting with diffuse cystic lesions, mimicking lymphangioleiomyomatosis (LAM).

CASE PRESENTATION: A 50-year-old non-smoking female with a history of asthma presented with an intermittent non productive cough. The cough responded well to albuterol. Pulmonary function tests revealed abnormal spirometry with mixed obstruction and restriction. Chest CT (Figure 1) unmasked innumerable small cystic lesions throughout the lungs. Based upon imaging, gender, and age, there was high suspicion for LAM. Serology including ANCA, ANA, ESR, and RF were normal. VATS biopsy for diagnosis was performed. Pathology unexpectedly revealed hypersensitivity pneumonitis [figure 2]. Oral steroids were started. Hypersensitivity panel was negative. Vigorous investigation of the patient’s environment uncovered mold in the basement. This was later removed. At follow up, spirometry showed mild obstruction with improvement and steroids were tapered.

DISCUSSION: In 1713 a medical professor described health hazards associated with 52 occupations, providing the first account of what is now called HP [1]. HP is caused by inhalation of an antigen to which the patient is sensitized and hyper-responsive. However, many exposed subjects develop an immune response with IgG antibodies to the antigen and lymphocytes in their lungs but never develop HP [2]. In a population-based study, the estimated annual incidence of interstitial lung disease was 30 per 100,000, with HP accounting for 2% [3]. In only a small percentage of patients with HP are lung cysts found. This case illustrates the need to expand initial evaluation of cystic lung disease to include HP.

CONCLUSIONS: In patients with HP, treatment includes avoiding the allergen and in some cases, systemic corticosteroids. While long term prognosis is generally perceived as good, some patients develop severe respiratory insufficiency. We demonstrate the need to consider a HP diagnosis in patients with cystic lung disease.

Reference #1: Ramazzini B. Diseases of workers. Wright WC, trans. New York, NY: Hafner, 1964; 243

Reference #2: Cormier Y, et al Significance of pre- cipitins and asymptomatic lymphocytic alveolitis: a 20-yr follow-up. Eur Respir J. 2004;23(4):523-525.

Reference #3: Coultas DB, Zumwalt RE, Black WC, Sobonya RE. The epidemiology of interstitial lung diseases. Am J Respir CritCare Med. 1994;150(4):967-972

DISCLOSURE: The following authors have nothing to disclose: Anita Rajagopal, Michael Shapiro

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