Chronic eosinophilic pneumonia
- Kevin K Brown, MD
Kevin K Brown, MD
- Professor of Medicine
- National Jewish Health, University of Colorado
- Talmadge E King, Jr, MD
Talmadge E King, Jr, MD
- Editor-in-Chief — Pulmonary, Critical Care, and Sleep Medicine
- Section Editor — Interstitial Lung Disease
- Dean, School of Medicine
- Vice Chancellor, Medical Affairs
- University of California San Francisco
Chronic eosinophilic pneumonia (CEP) is an idiopathic disorder characterized by an abnormal and marked accumulation of eosinophils in the interstitium and alveolar spaces of the lung [1,2].
The clinical manifestations, diagnosis, and treatment of chronic eosinophilic pneumonia will be reviewed here. The evaluation and differential diagnosis of eosinophilic lung diseases, in general, and an approach to acute eosinophilic pneumonia are presented separately. (See "Causes of pulmonary eosinophilia" and "Idiopathic acute eosinophilic pneumonia".)
CEP is a rare disorder. The incidence of CEP in an Icelandic registry was 0.23 cases/100,000 population per year between 1990 and 2004 . In registries of interstitial lung disease (ILD) in Europe, CEP accounted for 0 to 2.5 percent of cases of ILD . Women develop CEP about twice as often as men. A majority of patients are nonsmokers.
CEP typically affects patients in their 30s or 40s, although onset in childhood has been reported [5-7]. A history of atopy is found in 60 percent. Asthma precedes, accompanies, or subsequently occurs in over 50 percent of cases .
The disease has a gradual onset, with an interval of approximately four to five months between the appearance of initial symptoms and diagnosis . Typical symptoms include a productive cough (33 to 42 percent), fever (67 percent), breathlessness (57 to 92 percent), weight loss (57 to 75 percent), and night sweats [5,9].
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- Fujimori K, Shimatsu Y, Suzuki E, et al. [Chronic eosinophilic pneumonia complicated by bronchial asthma and diabetes mellitus successfully treated with suplatast tosilate and high-dose inhaled corticosteroid therapy]. Nihon Kokyuki Gakkai Zasshi 1999; 37:903.
- Kaya H, Gümüş S, Uçar E, et al. Omalizumab as a steroid-sparing agent in chronic eosinophilic pneumonia. Chest 2012; 142:513.
- Shin YS, Jin HJ, Yoo HS, et al. Successful treatment of chronic eosinophilic pneumonia with anti-IgE therapy. J Korean Med Sci 2012; 27:1261.
- Domingo C, Pomares X. Can omalizumab be effective in chronic eosinophilic pneumonia? Chest 2013; 143:274.
- Cazzola M, Mura M, Segreti A, et al. Eosinophilic pneumonia in an asthmatic patient treated with omalizumab therapy: forme-fruste of Churg-Strauss syndrome? Allergy 2009; 64:1389.
- Wechsler ME, Wong DA, Miller MK, Lawrence-Miyasaki L. Churg-strauss syndrome in patients treated with omalizumab. Chest 2009; 136:507.
- Golstein MA, Steinfeld S. Chronic eosinophilic pneumonia followed by Churg-Strauss syndrome. Rev Rhum Engl Ed 1996; 63:624.
- CLINICAL MANIFESTATIONS
- Pulmonary function tests
- DIFFERENTIAL DIAGNOSIS
- Initial treatment
- Assessing the response to therapy
- Treatment of disease relapse
- Duration of therapy
- Potential alternative therapies
- Prevention of glucocorticoid-related adverse effects
- SUMMARY AND RECOMMENDATIONS