ObjectiveTo explore the clinical characteristics of idiopathic hypereosinophilic syndrome (IHES), and improve the early diagnosis and treatment of such diseases.MethodsThe clinical diagnosis and treatment data were retrospectively analyzed from the patients with confirmed IHES hospitalized in China-Japan Friendship Hospital between September 2010 to May 2018.ResultsFifteen patients were included. There were 3 women and 12 men in the study, with an average age of 53.7±21.3 years. Eleven patients had respiratory problems, with an average course of 7 months. Most lesions occurred in both lungs. Patchy distribution, ground glass opacity, pleural effusion and mediastinal lymph node enlargement were common in the chest computed tomography. Serum total IgE was significantly increased. Four patients had other systems involved rather than respiratory system. One of them had digestive problems and another 3 had skin diseases. There was a significant increase in eosinophils in peripheral blood, bone marrow and histopathology, the same as eosinophils in sputum, pleural effusion, and bronchoalveolar lavage fluid. Hypoxemia was common in patients with respiratory problems. The blood eosinophil and total IgE were reduced after glucocorticoid treatment, and the hypoxemia was significantly improved.ConclusionsThe clinical symptoms, signs and image of chest computed tomography are not specific in IHES, so the rate of misdiagnosis and wrong diagnosis is high. This disease involves many organs or systems, so the pathological examination should be completed as soon as possible to make a clear diagnosis to prevent further damage. Glucocorticoid treatment is effective in this disease.
Since the launch of anti-interleukin 5 monoclonal antibodies (anti-IL-5 mAbs), they have been widely used in various disease fields. The classic indications include severe eosinophilic asthma (SEA), chronic rhinosinusitis with nasal polyps (CRSwNP), eosinophilic granulomatosis with polyangiitis (EGPA), hypereosinophilic syndrome (HES), and chronic obstructive pulmonary disease (COPD). Moreover, there is evidence suggesting that anti-IL-5 mAbs can also be used in the treatment of allergic bronchopulmonary aspergillosis (ABPA) and other uncommon eosinophil (EOS)-related diseases. To standardize the clinical application of anti-IL-5 mAbs by clinicians, the expert group formulated a consensus by extensive review of high-quality clinical research data and authoritative guideline documents. The consensus paper was written based on the current best clinical evidence and Chinese clinical practice experience, aiming to provide authoritative reference and practical guidance for clinical colleagues in the standardized clinical use of anti-IL-5 mAbs. The content of this consensus covers the mechanism of anti-IL-5 mAbs, dosing regimens for different diseases, management strategies, etc. It emphasizes the importance of individualized diagnosis and treatment guided by biomarkers, long-term standardized follow-up, and multidisciplinary team collaboration.
