Eosinophilic Enteritis: An Unlikely Cause of Exudative Eosinophilic Pleural Effusion

Abstract

INTRODUCTION: Exudative eosinophilic predominant pleural effusions are commonly encountered in daily practice. The more common conditions associated with this etiology of exudative effusions are blood or air in the pleural space. Also fungal or parasitic diseases are well known to cause this type of exudative effusions. Here we report the case of a patient with a history of eosinophilic enteritis that was diagnosed with an exudative effusion of eosinophilic predominance during a flare of her disease. CASE PRESENTATION: A 44 y/o female with a past medical history for eosinophilic enteritis and obstructive sleep apnea presented to the hospital complaining of severe abdominal pain, nausea, diarrhea and severe dyspnea. Similar gastrointestinal complaints a year earlier led to the diagnosis of eosinophilic gastroenteritis via push enteroscopy. Treatment with a tapering dose of prednisone and montelukast for one month led to resolution of her symptoms after the initial diagnosis was made. The patient had been in her normal state of health a week prior. Her respiratory complaints were dyspnea at rest and severe dyspnea with mild exertion. No history of recent travel, sick contacts, or previous atopic or pulmonary disease. Her vital signs were significant for hypoxemia at rest to 86% on room air. Her exam was positive for decreased air entry in the right hemithorax and dullness to percussion over the right hemithorax. Chest X-ray showed a large right pleural effusion and CT scan of the thorax showed a very large right sided pleural effusion with collapse of the right lower and middle lobes. The patient had a thoracentesis with drainage of 1500cc of serosanguinous fluid. The pleural fluid analysis showed a leukocyte count of 3550 with 78% predominance of eosinophils. Additional pleural fluid studies showed a total protein of 5.2 gm/dL and LDH of 203 IU/L, pH of 7.36. Serum studies showed a total protein of 8.2 gm/dL, LDH of 241 IU/L. Pleural fluid cytology was negative for malignancy. Serology for parasitic disease and stool studies were negative. A work up for vasculitis including ANA, ANCA panels are negative. Previous colonoscopy was also negative for malignancy. Drainage of her pleural effusion and treatment IV steroids followed by a tapering dose of steroids with montelukast led to improvement of her symptoms. The patient was seen at follow up in the outpatient pulmonary clinic 9 days later with a repeat chest x-ray which showed complete resolution of the pleural effusion. Her respiratory and gastrointestinal symptoms had resolved as well. DISCUSSION: Eosinophilic enteritis is a rare condition with only about 300 cases reported. It is characterized by eosinophilic infiltration of the gastrointestinal tract consisting of 20 or more eosinophils per high-power field and usually with absence of multiorgan involvement with eosinophilia. Also there cannot be an identifiable cause for eosinophilia like parasitic infection or malignancy. It is often accompanied by a history of atopy or allergies especially to food. Eosinophilic ascites and eosinophilic pleural effusions have been described in patients with eosinophilic enteritis. The etiology for the formation of the pleural effusions is currently unknown. Treatment of the underlying condition in this case showed dramatic resolution of the patient's effusion and led to prompt symptom improvement. CONCLUSIONS: Exudative pleural effusion can be seen in patients with this eosinophilic enteritis. The effusion can be large as the one seen in our patient and may cause significant respiratory compromise. Treatment of the underlying condition with steroids and leukotriene inhibitors are key to the management of these effusions.