**Chronic Eosinophilic Pneumonia Medical Guide (Introduction)**
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**Chronic Eosinophilic Pneumonia (Alternative names: Chronic Myelogenous Pneumonia, Chronic Eosinophilic Pneumonia, Persistent Eosinophilia, Persistent Pulmonary Eosinophilic Infiltration)**
What is chronic eosinophilic pneumonia?
Chronic eosinophilic pneumonia (CEP), also known as persistent eosinophilia or chronic myelogenous pneumonia (chronic eosinophilic pneumonia, CEP), has a longer course than simple eosinophilia, usually lasting from 2 to 6 months, and sometimes even over a year. The symptoms are also more severe.
What are the manifestations of chronic eosinophilic pneumonia? Are there any special symptoms?
What are the early symptoms of chronic eosinophilic pneumonia?
This disease can occur at any age, but the peak incidence is between 30-40 years old, with women being almost twice as likely to be affected as men. Most cases are Caucasians, and one-third to one-half of patients have a history of allergic rhinitis or nasal polyps. Additionally, two-thirds of patients have adult-onset asthma or other respiratory symptoms. Clinically, it presents subacutely, with common symptoms including low-grade fever, excessive night sweating, moderate weight loss, cough, slight mucus production, and about 2/9 of patients experiencing mild hemoptysis. Eventually, patients may develop progressive dyspnea associated with episodic asthma. A few patients present with acute severe respiratory failure or ARDS.
Based on medical history, disease progression, wheezing in both lungs, increased eosinophils in peripheral blood, and chest X-ray shadows, a clinical diagnosis can be made. For atypical cases, lung biopsy for pathological examination may be necessary to confirm the diagnosis. If needed, trial treatment with prednisone can assist in diagnosis.
After understanding the manifestations of chronic eosinophilic pneumonia, what tests should we perform?
What tests should be conducted for chronic eosinophilic pneumonia?
- Leukocytosis > 10 × 10^9/L.
- 60%-90% of patients have increased eosinophils in peripheral blood (>6%), although the absence of eosinophils does not rule out the disease. Numerous eosinophils can be found in sputum. Increased erythrocyte sedimentation rate (>20 mm/h). One-third of cases show elevated IgE levels in the blood.
- Abnormalities in pulmonary function tests vary with the stage of the disease. Typically, moderate to severe restrictive ventilatory dysfunction and decreased DLCO are observed.
- Elevated alveolar-arterial oxygen gradient; if accompanied by asthma, obstructive changes may be present.
- Chest X-ray shows progressive infiltrates with increasing density near the pleura, unclear margins, non-segmental, subsegmental, and lobar distribution, mostly located in the outer two-thirds of the lungs. The hilar region appears more transparent, hence the term "reversed halo sign." Shadows tend to recur in the same location. After prednisone treatment, shadows resolve quickly. Unlike Löffler's syndrome, CEP lung infiltrates are non-migratory, and pleural effusion is rare. Atypical X-ray findings include nodular infiltration and diffuse ground-glass opacities indicating alveolar filling.
- Chest CT scans can be performed for clinically suspected cases with atypical X-ray findings. CT typically shows typical dense areas and peripheral localized alveolar consolidation within the first few weeks of symptom onset. When symptoms persist for more than two months, cord-like and band-like opaque areas appear, along with enlarged mediastinal lymph nodes.
After confirming the diagnosis through testing, how should we proceed with treatment?
Precautions before treating chronic eosinophilic pneumonia:
**(I) Treatment**
Prednisone is the primary treatment for CEP. Most cases respond well to prednisone (initial dose of 40 mg/day), with fever subsiding within 6 hours, improvement in dyspnea, cough, and eosinophilic infiltration within 24-48 hours, resolution of hypoxemia within 2-3 days, and radiographic improvement within 1-2 weeks. Rapid improvements can occur within 2-4 days. Complete symptom relief occurs within 2-3 weeks, and X-rays return to normal within two months. Once symptoms improve and pulmonary findings resolve, prednisone can be gradually tapered (approximately every 10-14 days), with a total treatment duration of 4-6 months.
**(II) Prognosis**
The prognosis for CEP is generally good, but fatalities can occasionally occur. Untreated patients rarely experience spontaneous remission, whereas those treated with corticosteroids have significantly reduced mortality rates. However, 58%-80% of patients relapse when prednisone is tapered too quickly or interrupted. Relapses often occur in the same anatomical locations, requiring treatment for 1-2 years. About 25% of patients require long-term maintenance doses (2.5-10 mg/day) until the disease resolves. Despite no clear indicators predicting recurrence or the need for prolonged treatment, short-term (1-3 months) treatment often leads to relapse, sometimes multiple times. However, re-initiating prednisone remains effective.
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