Chronic Eosinophilic Pneumonia: Adjunctive Therapy With Inhaled Steroids

Abstract

a b s t r a c tIdiopathic 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 2e4 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/mm 3 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 30e50% 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.

A Case of Chronic Eosinophilic Pneumonia Associated With the Use of Weight Loss Combination Medication Naltrexone-Bupropion

“…The high recurrence rate of CEP can lead to steroid dependence, provoking research into other possible treatment options. One case report outlines a woman who maintained remission on inhaled steroids following systemic steroid cessation [ 11 ], but further research is necessary to conclude whether inhaled steroids may contribute to remission maintenance. There are few case reports that have reported that the anti-immunoglobulin E (anti-IgE) antibody omalizumab was used to treat cases of steroid-dependent eosinophilic pneumonia [ 12 , 13 ].…”

Recurrent pneumonia with persistent peripheral eosinophilia: Idiopathic chronic eosinophilic pneumonia

“…Leukocytosis and peripheral eosinophilia are present in more than 90% of cases and may help signify an alternative diagnosis other than bacterial/viral pneumonia. There are no absolute diagnostic criteria for ICEP but diagnosis is made based on respiratory symptoms for at least 2 weeks, presence of multilobar peripheral ground‐glass opacities, and eosinophilia >40% on BAL or peripheral eosinophilia >1000/mm 3 in the absence of other eosinophilic lung diseases 1,3 . Other such causes of eosinophilic pneumonia that need to be excluded prior to diagnosis include but not limited to drug‐induced eosinophilic pneumonia, eosinophilic granulomatosis with polyangiitis (EGPA, also known as Churg Strauss), allergic bronchopulmonary aspergillosis (ABPA), and fungal and parasitic infections depending on exposure history 3 …”

“…Idiopathic chronic eosinophilic pneumonia, also known by the eponym Carrington disease, is a rare eosinophilic lung disease that accounts for less than 3% of cases of interstitial lung disorders 1 . Patients present with ill‐defined symptoms of dyspnea, cough, wheezing, and fatigue over several months as well as the presence of eosinophilia and opacities on chest imaging 2,3 . Once diagnosed, ICEP typically responds well to corticosteroid therapy.…”