Four Cases Report Of Chronic Eosinophilic Pneumonia And Literatures Systemic Analysis

Objective: To summarize the clinical features of patients with Chronic eosinophilic pneumonia(CEP)on our study and hope it would be helpful in improving the diagnosis and therapy.Methods: The clinical feature,treatment and prognosis of 4 patients with CEP admitted to the First Affiliated Hospital of Guangxi Medical University from January 2008 to September 2016 are retrospectively analysised.By retrieving the literatures published on the databases such as the Chinese hownet Wanfang and Pubmed,we analysis systematically its clinical manifestation and therapy.Results:(1)Data in four cases: ? There were 2 men and 2 wemen,rang26~67yr,2 of them had a tobacco smoking history.The onset of this study was Subacute or chronic,with 1m~4yr between onset symptom and the diagnosis.The clinical manifestations were not specific and without extrathoracic manifestations.Cough was presented in all patients,sputum production and exertional dyspnea in 2 cases,wheezing,chest tightness and chest pain were in one patien respectively.Fever,fatigue,anorexia and weight loss were in one case.One pation heard wet rale.? All patients had performed autoantibodies,conventional biochemical and muscle enzymes,parasites,pathogens and tumor antigens,T cell subsets,complement and electrocardiogram examination.So any known causes of eosinophilic lung disease,multi-systemic diseases,allergic diseases,and cancers were excluded.All had been respectively misdiagnosed as asthma,tuberculosis,bronchitis,and pneumonia for 1m~2yr.?3 cases had bloodeosinophilia(1.01~3.34×10~9/L).Some examination presented increasing included peripheral blood white blood cell and mild anemia in 2,ESR and CRP in 3,and hypoxia in 2.(4)All patients were performed bronchoscopy showing mucosal hyperemia in 3,purulent secretion in 2.In BALF,eosinophilia was demonstrated in one(30%),another case(0.0217 x 10~9/L).TBLB interstitial and alveolar of 4 cases were infiltrated with EOS,lymphocytic also were found.2 cases performanced pulmonary interstitial swelling and mild hyperplasia of alveolar epithelium.(5)CTshowed ground glass opacity in 4,patchy consolidation in 3,inhomogeneous dense infiltration in 2,cavitationin in 2,funicular and grid changes in one.Migration opacities were recorded in 2 patients.All were demonstrating bilateral opacities.Lesions were limited to upper-middle part of lung,peripher and alunder pleura in 3 cases respectively.(6)Pulmonary function test were performed in 3 patients,2 of them had diffuse defect.Mixed ventilatory defect and restrictive in one respectively.(7)All were treated with OCST,showing subjective symptom rapidly improved within 1w.And the peripheral blood eosinophils recovered in 1w~2w.Chest CT of four were absorbed in 1w~2m,3of them show that opacities were disappeared.There was 2 relapses in one case with migration opacities.Just one had reviewed pulmonary function test showing diffuse defect improved.The visiting time in this group was 10m~8yr.(2)A systemic analysis of related literatures:Since 1975,Only 70 cases with detailed information were reported in the world.BALF or lung biopsy confirmed EOS infiltration,diagnosis of CEP were made when secondary,tumor,autoimmune diseases,extrapulmonary organs infiltrated with EOS and other interstitial lung disease were excluded,and most of them were case report.(1)General condition:the patients were rang 1~79yr and M:F=26:35,and there was a peak incidence between the ages of 40 yr and 60 yr,with the average 50.13 yr.19 cases had been misdiagnosed and 14 cases had asthma previously.Only 3 had acute respiratory failure,needed mechanical ventilation.The most common clinical manifestations were cough,shortness of breath and sweezing,and fever,night sweat,weight loss were allowed.There wasn't extrapulmonary complaint.About physical examination,pulmonary rales were heard in 22,and clubbing in one.? 87.7persent had eosinophilia in peripheral blood.54 cases did bronchoscopy examination showing mucinous sputum in 8,and white nodules in 2.Eosinophilia were found in BALF(average 49.1%).Lung biopsy were performed in 50 cases,showed that alveolar and interstitial infiltrated with EOS.6 of them hadmild fibrosis.34 cases provided pulmonary function test results,and 68.4persent had diffuse defect.The ventilatory function could be obstructive,restrictive,mixed or normal.47.1 persent indicated obstruction which were more common in patients with asthma history.?About Radiologic imaging,bilateral multiple patchy solid,ground glass shadow in upper lobule and peripheral were common.(4)In addition to 4 cases treated with ICS in the beginning,and than changing to OCST because of getting worse.Otherwise 66 patients treated with OCST on the initial dose of prednisone 0.3~2.0mg/Kg.d with 9m on average,the longest 11 years.(5)Except for 1 patien died due to coronary heart disease,the prognosis of CEP was good.They relapsed for 12 times while glucocorticoids were being weaned or tapered,8 of them occurred in the begining treatment for less than 6 months.After relapse,retreatment with OCST was also sensitived.Conclusion:(1)Lung were infiltrated with eosinophils,when secondary factors,extrapulmonary organs impairmened were excluded by taking routine biochemical,pathogenicity,immune antigen antibody examination,there weren't knowm causes.So CEP should be considered.(2)Bone marrow examination of fusion gene help to exclude clonal myeloproliferative disease,it should be necessary or not for the diagnose of CEP is not clear and need further study.(3)CEP has a long duration with good prognosis,often happens 0n 40~69yr with history of cough,shortness of breath and sweezing,fever,night sweat and weight loss,without extrapulmonary symptoms,always with Misdiagnosis on clinical.(4)More than 60 persent patients' chest imaging showed bilateral multiple patchy solid,ground glass shadow in upper lobule and peripheral.16.6 persent showed migration opacities.(5)OCST is first-line therapy of CEP,and the initial dose on prednisone 0.5~1mg/kg.d is recommended for 9m,not less than 6months.It always relapses when corticosteroids tapering and wean within 6months.While it's still responsive to corticosteroids.It should decrease slowly,prolong the duration and be individualized.