Chronic eosinophilic pneumonia: Adjunctive therapy with inhaled steroids

Abstract

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.

Recurrent pneumonia with persistent peripheral eosinophilia: Idiopathic chronic eosinophilic pneumonia

“… 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 …”

“…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 Once diagnosed and treatment is started, patients typically respond well to steroids with improvement in symptoms and rapid resolution of eosinophilia.…”

“…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 Once diagnosed and treatment is started, patients typically respond well to steroids with improvement in symptoms and rapid resolution of eosinophilia. However, biologics also have a role in cases refractory to corticosteroids 2,4 or as steroid-sparing agents.…”

“… 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. However due to the broad differential of diseases with similar respiratory symptoms and imaging findings, ICEP can be mistaken for infectious etiologies.…”

10-Year Follow-Up of Frequently Relapsed Chronic Eosinophilic Pneumonia Starting at 15 Years Old; Attempts to Treat with Inhaled Corticosteroid (A Case Report)

“…But other group considered inhaled steroids not effective [8]. Chan et al [9] reported a patient who underwent treatment with systemic corticosteroids followed by inhaled steroids and who remained in remission for 2 years. We tried 5 kinds of inhaled steroids during the active and the remission phases of eosinophilic pneumonia to avoid the adverse effects associated with systemic administration of steroids, but we could not achieve sufficient efficacy using inhaled steroids.…”