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Idiopathic chronic eosinophilic pneumonia.
Eric Marchand1, Jean-François Cordier
1Service de Pneumologie, Cliniques Universitaires de Mont-Godinne, Université Catholique de Louvain, B-5530 Yvoir, Belgium. eric.marchand@pneu.ucl.ac.be
Orphanet Journal of Rare Diseases
|May 26, 2006
Summary
Idiopathic chronic eosinophilic pneumonia (ICEP) is a rare lung disorder. Corticosteroids are the primary treatment, offering dramatic symptom relief but often requiring long-term use due to frequent relapses.
Area of Science:
- Pulmonology
- Respiratory Medicine
- Immunology
Background:
- Idiopathic chronic eosinophilic pneumonia (ICEP) presents with respiratory and systemic symptoms.
- Diagnosis involves identifying eosinophilia in blood or bronchoalveolar lavage (BAL) fluid, often with a higher eosinophil to lymphocyte ratio in BAL.
- ICEP is rare, of unknown etiology, affects all ages (rarely children), and is more common in women, with a significant history of asthma in affected individuals.
Purpose of the Study:
- To describe the characteristics, diagnosis, and treatment of Idiopathic Chronic Eosinophilic Pneumonia (ICEP).
Main Methods:
- Review of clinical presentation, diagnostic criteria, and treatment outcomes for ICEP.
- Emphasis on diagnostic role of blood and BAL eosinophilia.
- Evaluation of corticosteroid response and long-term management.
Main Results:
- ICEP is characterized by eosinophilia (often >1000/mm3) and pulmonary infiltrates.
- BAL eosinophilia supports diagnosis in cases of low blood eosinophilia.
- Systemic corticosteroids provide rapid symptom improvement, but relapses are common upon dose reduction.
Conclusions:
- ICEP is a distinct clinicoradiological entity defined by eosinophilic lung infiltration.
- Corticosteroid therapy is effective but frequently necessitates long-term management.
- Further research is needed to understand the underlying causes and optimize treatment strategies for ICEP.