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Chronic idiopathic eosinophilic pneumonia
Chronic idiopathic eosinophilic pneumonia (CIEP) is a rare inflammatory lung disease characterized by eosinophilic infiltration of the lung parenchyma, leading to subacute or chronic respiratory symptoms.
Prevalence
1 / 100 000
N/A
US Estimated
N/A
Europe Estimated
Age of Onset
All ages
ICD-10
J82.81
Inheritance Pattern
Autosomal dominant
Autosomal recessive
Mitochondrial/Multigenic
X-linked dominant
X-linked recessive
Rare View
Chronic Idiopathic Eosinophilic Pneumonia (CIEP) is a rare lung disorder characterized by the accumulation of eosinophils, a type of white blood cell, in the lungs.
5 Facts you should know
FACT
Chronic Idiopathic Eosinophilic Pneumonia (CIEP) is a rare lung disorder characterized by the infiltration of eosinophils into the lungs, leading to respiratory symptoms and lung inflammation
FACT
It typically presents with cough, shortness of breath, fever, night sweats, and chest pain, resembling symptoms of pneumonia, often persisting for weeks to months
FACT
Diagnosis involves clinical assessment, chest imaging revealing infiltrates, and bronchoalveolar lavage demonstrating increased eosinophils (>25%) in the lungs, excluding other causes of eosinophilic lung diseases
FACT
Treatment primarily involves corticosteroids as the first-line therapy, leading to rapid improvement in symptoms and lung function in most cases. Relapses upon tapering or discontinuation of steroids are common
FACT
Long-term management may require a gradual reduction in steroid dosage, sometimes necessitating low-dose maintenance therapy, with careful monitoring for relapses and potential complications
Interest over time
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Common signs & symptoms
Subacute cough
Dyspnea
Wheeze/Asthma history
Fatigue
Fever/Night sweats
Weight loss
Current treatments
Systemic corticosteroids
mainstay (often rapid clinical/radiographic response; relapses common with taper)
For recurrent/steroid-dependent cases
steroid-sparing approaches may be used (specialist-guided; evidence evolving)
References:
Bento J, Botelho C, Souto Moura C, Morais A. Chronic eosinophilic pneumonia. <i>Acta Med Port.</i> 2010;23(6):1133–1140. doi:10.20344/amp.736 — Description of clinical features, eosinophilia, imaging patterns, and response to corticosteroids in chronic eosinophilic pneumonia. Teixeira N, Santos MI, Pedro F, et al. Idiopathic chronic eosinophilic pneumonia. Cureus. 2021;13(3):e14047. doi:10.7759/cureus.14047 — Detailed case description and discussion of eosinophilic infiltration, imaging, diagnosis, and steroid responsiveness. Idiopathic chronic eosinophilic pneumonia. Orphanet J Rare Dis. 2006;1:11. doi:10.1186/1750-1172-1-11 — Review of the disease’s clinical presentation, diagnostic criteria, epidemiology, and management with corticosteroids. Case report: Chronic eosinophilic pneumonia presenting with ipsilateral pleural effusion. J Med Case Rep. 2016;10:227. doi:10.1186/s13256-016-1005-5 — Case report illustrating typical presentation and dramatic response to steroids. Lee J, Rivera MP. Chronic eosinophilic pneumonia. MSD Manual Professional Edition. Reviewed Jun 2025 — Clinical perspectives on diagnosis, radiographic features, eosinophilia, and management.