Clinical Features Of 7 Cases Of Idiopathic Eosinophilic Syndrome With "Asthma" As The Initial Symptom

OBJECTIVE:This study summarizes the clinical features of idiopathic hypereosinophilic syndrome with "asthma" as the initial symptom in order to improve its diagnosis and therapy.METHODS:We retrospectively analyzed the clinical therapy and prognosis of 7 cases of IHES with "asthma" as the initial symptom to this hospital from January 2010 to December 2015.RESULTS: (1)Our study included 1 male and 6 female .The median age was 50 years. The duration of disease was 12(range 1,24) months.(2)All patients presented cough and wheezing ,which were the first performance. There was fearless cold, fever, weight loss or other systemic symptoms. (3)5 cases had increased white blood cell, 2 cases were normal. The average of white blood cell was(12. 20 ± 4. 34) X 109/L. A great number of eosnophils in the peripheral blood could be found in all patients, which had the average of (5.0±4.25)×109/L. The percentage of eosinophils was significantly increased in all patients, which had the average of( 37 ± 1. 71)% and the highest proportion of 65. 7%. All patients had Hypoxemia without carbon dioxide retention.(4)Chest CT mainly perform patchy shadows and ground—glass opacity. Pulmonary function can be expressed as obstructive ventilation dysfunction ,mixed ventilation dysfunction, diffuse dysfunction and dilation test positive. A large number of eosinophil infiltration in pulmonary interstitial in one case,(5)The percentage of eosinophils was significantly increased in the bone marrow, which had the highest proportion of 33.5%. FIPILI-PDGFRa genes were negative in all patents.(6)All patients were treated with corticosteroids (or combined with immunosuppressants therapy), as well as the specification of inhaling corticosteroids and Bronchodilators. Six patients were treated with a single does of glucocorticoid . One patient was received glucocorticoid, and was conjuncted with Immunosuppressants therapy. With a follow-up period of 7 months(range,2-36months),7 cases were relieved. But 4 cases repeated episode after the withdrawal of corticosteroids. and immunosuppressantst after 2 to 12 months. Three patients have been still taking glucocorticoid, one control well, the others repeated episode.CONCLUSIONS: (1) (1) Idiopathic hypereosinophilic syndrome with"asthma" as the initial symptom was more common in female. It was expressed as "asthma", which was persistent and refractory in the early stage of the disease. And it was responded poor to inhalation therapy. A large number of mature eosinophils infiltration in the peripheral blood,bone marrow and lung. The prominent organ involvement was mainly limited to the lung, but systemic symptoms and other organs involved light. (2) Chest CT mainly perform patchy shadows and ground—glass opacity, pulmonary function can be expressed as ventilation dysfunction and diffuse dysfunction. (3) Glucocorticoids are the mainstay of therapy and effective fist-line agent of the IHES. It can be combinated with inhaling corticosteroids and Bronchodilators. Most patients repeated episode in the reduction of glucocorticoids. Their airway obstruction can not be completely reversible, and the pulmonary function are difficult to return to normal. It may be conjunction with Immunosuppressants when it is necessary.