Chronic eosinophilic pneumonia: Adjunctive therapy with inhaled steroids

Idiopathic chronic eosinophilic pneumonia (ICEP) is a rare form of diffuse parenchymal lung disease first identified by Carrington et al. in 1969. It is characterized by the presence of constitutional and respiratory symptoms with associated peripheral opacities on imaging and elevated serum and/or bronchoalveolar eosinophilia. Although data is limited regarding etiology or prevalence, it is known that ICEP has a 2:1 female: male predominance and typically affects non-smokers. Diagnosis rests on the clinical constellation of respiratory symptoms of at least 2-4 weeks duration, the presence of diffuse pulmonary alveolar consolidation, classically described as the "photographic negative of pulmonary edema", the presence of eosinophils ≥40% on bronchoalveolar lavage or ≥1000/mm³ eosinophils on peripheral blood and the exclusion of other known causes of eosinophilic lung diseases such as drugs, toxins, fungi, parasites, and collagen-vascular disorders. A dramatic response is achieved with systemic corticosteroids, which is typically dosed over 6 months to 1 year. Despite this response, approximately 30-50% of patients will relapse upon cessation of steroids or during the taper. Although these patients respond well to another trial of steroids, the side effects of long term steroids are well known, including osteoporosis, diabetes, hypertension and cataracts. Inhaled corticosteroids as monotherapy has been trialed in the past without success. However, we report a case of a patient who underwent treatment with systemic corticosteroids followed by inhaled steroids who has remained in remission for 2 years.