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Chronic eosinophilic pneumonia: causes, symptoms, diagnosis, treatment
Last reviewed: 23.04.2024
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Chronic pulmonary eosinophilia (prolonged pulmonary eosinophilia, Lera-Kindberg syndrome) is a variant of simple pulmonary eosinophilia with the existence and recurrence of eosinophilic infiltrates in the lungs for more than 4 weeks. Chronic eosinophilic pneumonia is characterized by chronic pathological accumulation of eosinophils in the lung.
Prevalence and incidence of chronic eosinophilic pneumonia (HEP) are unknown. Chronic eosinophilic pneumonia is believed to be an allergic diathesis. Most patients are non-smokers.
[What causes chronic eosinophilic pneumonia?
The causes of this form of pulmonary eosinophilia are the same as that of Leffler's syndrome, but in addition, the cause of the disease can be tumors (stomach, thyroid, lung), hemoblastosis, systemic vasculitis and systemic connective tissue diseases.
The main pathogenetic factors are the same as for simple pulmonary eosinophilia.
Symptoms of chronic eosinophilic pneumonia
Chronic eosinophilic pneumonia often develops with lightning speed: there is a cough, an increase in body temperature, progressive dyspnoea, weight loss, wheezing and night sweats. Bronchial asthma accompanies or precedes the disease in more than 50% of cases.
Where does it hurt?
What's bothering you?
Diagnosis of chronic eosinophilic pneumonia
The diagnosis requires the exclusion of infectious causes and is based on the analysis of clinical manifestations, the results of blood tests and chest radiography. Often found eosinophilia of peripheral blood, very high ESR, iron deficiency anemia and thrombocytosis. On the roentgenogram of chest organs, bilateral infiltrates are detected in the lateral or subpleural zones (approximately 60% of cases), usually in the middle and upper parts of the lung, described as a "negative" pulmonary edema; this picture is pathognomonic (although it occurs in <25% of patients). With CT, similar changes are detected in virtually all cases. Eosinophilia of bronchoalveolar lavage (> 40%) is a reliable sign of chronic eosinophilic pneumonia; Studies of bronchoalveolar lavage in dynamics can help control the course of the disease. Histological examination of lung biopsy reveals interstitial and alveolar eosinophils and histiocytes, including multinucleated giant cells and obliterating bronchiolitis with organizing pneumonia. Fibrosis is minimal.
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What tests are needed?
Treatment of chronic eosinophilic pneumonia
With chronic eosinophilic pneumonia, the effectiveness of intravenous or oral glucocorticoids is high; the absence of a response points to another diagnosis. Initial treatment of chronic eosinophilic pneumonia consists in the appointment of prednisolone (in a dose of 40 to 60 mg, once a day). Clinical recovery is often remarkably rapid, perhaps within 48 hours. A complete resolution of clinical manifestations and radiologic changes occurs within 14 days in most patients and 1 month in almost all patients. Therefore, assessment of the dynamics of these indicators is a reliable and effective means of monitoring the effectiveness of therapy. Although CT is more sensitive in detecting X-ray changes, its advantages in assessing the dynamics of the process are not shown. The number of eosinophils in the peripheral blood, ESR and IgE concentration can also be used to monitor the clinical course of the disease against the background of the treatment. However, not all patients have pathological changes in the results of laboratory tests.
Clinical or radiologic relapse is noted in 50-80% of cases after discontinuation of therapy or, less often, with a decrease in the dose of glucocorticoids. Relapse can develop in months and years after the initial episode of the disease. Thus, the treatment of chronic eosinophilic pneumonia with glucocorticoids sometimes continues indefinitely. Inhaled glucocorticoids (for example, fluticasone or beclomethasone in a dose of 500 to 750 micrograms twice a day) are probably effective, especially with a decrease in the maintenance dose of oral glucocorticoid.
Chronic eosinophilic pneumonia sometimes leads to physiologically significant and irreversible pulmonary fibrosis, although lethal outcomes are extremely rare. Relapse probably does not indicate a lack of treatment effect, a worse prognosis or a more severe course. Patients continue to respond to glucocorticoids, as in previous episodes. A fixed airflow restriction may be noted in some recovered patients, but these disorders are usually of limited clinical significance.