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 and Critical Care 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].
- Allen JN, Davis WB. Eosinophilic lung diseases. Am J Respir Crit Care Med 1994; 150:1423.
- Cordier J-F, Cottin V. Eosinophilic pneumonias. In: Interstitial Lung Disease, 5th, Schwarz MI, King TE, Jr (Eds), People's Medical Publishing House-USA, Shelton, CT 2011. p.833.
- Sveinsson OA, Isaksson HJ, Gudmundsson G. [Chronic eosinophilic pneumonia in Iceland: clinical features, epidemiology and review]. Laeknabladid 2007; 93:111.
- Thomeer MJ, Costabe U, Rizzato G, et al. Comparison of registries of interstitial lung diseases in three European countries. Eur Respir J Suppl 2001; 32:114s.
- Marchand E, Reynaud-Gaubert M, Lauque D, et al. Idiopathic chronic eosinophilic pneumonia. A clinical and follow-up study of 62 cases. The Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM"O"P). Medicine (Baltimore) 1998; 77:299.
- Nathan N, Guillemot N, Aubertin G, et al. Chronic eosinophilic pneumonia in a 13-year-old child. Eur J Pediatr 2008; 167:1203.
- Cakir E, Aksoy F, Cakır FB, Ertem T. Chronic eosinophilic pneumonia with mucous plugs in a child. Pediatr Pulmonol 2010; 45:1040.
- Marchand E, Etienne-Mastroianni B, Chanez P, et al. Idiopathic chronic eosinophilic pneumonia and asthma: how do they influence each other? Eur Respir J 2003; 22:8.
- Jederlinic PJ, Sicilian L, Gaensler EA. Chronic eosinophilic pneumonia. A report of 19 cases and a review of the literature. Medicine (Baltimore) 1988; 67:154.
- Marchand E, Cordier JF. Idiopathic chronic eosinophilic pneumonia. Orphanet J Rare Dis 2006; 1:11.
- Valent P, Klion AD, Horny HP, et al. Contemporary consensus proposal on criteria and classification of eosinophilic disorders and related syndromes. J Allergy Clin Immunol 2012; 130:607.
- Matsuse H, Shimoda T, Fukushima C, et al. Diagnostic problems in chronic eosinophilic pneumonia. J Int Med Res 1997; 25:196.
- Durieu J, Wallaert B, Tonnel AB. Long-term follow-up of pulmonary function in chronic eosinophilic pneumonia. Groupe d'Etude en Pathologie Interstitielle de la Société de Pathologie Thoracique du Nord. Eur Respir J 1997; 10:286.
- Gaensler EA, Carrington CB. Peripheral opacities in chronic eosinophilic pneumonia: the photographic negative of pulmonary edema. AJR Am J Roentgenol 1977; 128:1.
- Jeong YJ, Kim KI, Seo IJ, et al. Eosinophilic lung diseases: a clinical, radiologic, and pathologic overview. Radiographics 2007; 27:617.
- Zimhony O. Photographic negative shadow of pulmonary oedema. Lancet 2002; 360:33.
- Johkoh T, Müller NL, Akira M, et al. Eosinophilic lung diseases: diagnostic accuracy of thin-section CT in 111 patients. Radiology 2000; 216:773.
- Furuiye M, Yoshimura N, Kobayashi A, et al. Churg-Strauss syndrome versus chronic eosinophilic pneumonia on high-resolution computed tomographic findings. J Comput Assist Tomogr 2010; 34:19.
- Danel, C, Israel-Biet, D, Costabel, U, et al. The clinical role of BAL in rare pulmonary diseases. Eur Respir Rev 1991; 2:83.
- Marcq M, de Muret A, Letourmy JM, et al. [Carrington's disease without eosinophils in the bronchial lavage]. Rev Mal Respir 2007; 24:896.
- Taniguchi H, Kadota J, Abe K, et al. [Eosinophilic pneumonia without eosinophilia in BALF or peripheral blood and diagnosed by open lung biopsy]. Nihon Kokyuki Gakkai Zasshi 1999; 37:796.
- Mochimaru H, Kawamoto M, Fukuda Y, Kudoh S. Clinicopathological differences between acute and chronic eosinophilic pneumonia. Respirology 2005; 10:76.
- Lee JY, Choi H, Chon GR. Allergic bronchopulmonary aspergillosis mimicking relapsing chronic eosinophilic pneumonia in non-asthma patient. Allergol Int 2014; 63:133.
- Yoshida K, Shijubo N, Koba H, et al. Chronic eosinophilic pneumonia progressing to lung fibrosis. Eur Respir J 1994; 7:1541.
- Ebara H, Ikezoe J, Johkoh T, et al. Chronic eosinophilic pneumonia: evolution of chest radiograms and CT features. J Comput Assist Tomogr 1994; 18:737.
- Naughton M, Fahy J, FitzGerald MX. Chronic eosinophilic pneumonia. A long-term follow-up of 12 patients. Chest 1993; 103:162.
- Minakuchi M, Niimi A, Matsumoto H, et al. Chronic eosinophilic pneumonia: treatment with inhaled corticosteroids. Respiration 2003; 70:362.
- Lavandier M, Carré P. Effectiveness of inhaled high-dose corticosteroid therapy in chronic eosinophilic pneumonia. Chest 1994; 105:1913.
- 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