Sevim Tülin, Aksoy Emine, Akyıl Fatma Tokgöz, Ağca Meltem Çoban, Kongar Nilüfer Aykaç, Özşeker Ferhan
Clinic of Chest Diseases, Süreyyapaşa Chest Diseases and Chest Surgery Training and Research Hospital, İstanbul, Turkey.
Department of Chest Diseases, Gayrettepe Florence Nightingale Hospital, İstanbul, Turkey.
Turk Thorac J. 2015 Oct;16(4):172-179. doi: 10.5152/ttd.2015.4614. Epub 2015 Oct 1.
Eosinophilic lung diseases are a rare group of heterogeneous diseases characterized by the increase of the eosinophil ratio in airways and lung parenchyma. In our clinic, patients diagnosed with eosinophilic lung disease were evaluated with their clinical features and prognoses.
In our clinic, 12 cases that were diagnosed and followed up for eosinophilic lung disease [eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome) (n=4), chronic eosinophilic pneumonia (CEP) (n=7), and simple pulmonary eosinophilia (Löffler's syndrome) (n=1)] were retrospectively evaluated.
Of the 12 cases, 8 were females, and the average age was 43 (28-72) years. All cases were undergoing bronchodilator therapy with asthma diagnosis (2 months-40 years). Additionally, 4 of the cases had sinusitis, and 1 had allergic rhinitis. The most common complaints of the patients were difficulty in breathing and coughing, and the duration of complaints was a median of 2 months. Peripheral eosinophilia and total IgE elevation were present during the admission of all cases; additionally, leucocyte elevation was recorded in 10 of them, anemia in 4 of them, and thrombocytosis in 4 of them. Moreover, 43% of the recorded DLCO values were lower than normal. Of the 10 cases that underwent bronchoalveolar lavage (BAL), the eosinophil ratio was above 25% in 7 subjects. Of the 8 cases that underwent transbronchial biopsy, eosinophil-involving infiltration was detected in 6 subjects. Additional findings in cases diagnosed with EGPA were nasal polyposis (n=1), sinusitis (n=2), polyneuropathy (n=1), cardiac involvement (n=2), and skin involvement in biopsy (n=1). Spontaneous recovery was observed in the patient diagnosed with simple pulmonary eosinophilia during the follow-up that was performed based on the history and laboratory and BAL results of the patient. Prednisolone treatment was started for all cases, except for simple pulmonary eosinophilia, and their controls were performed. Relapse was observed in eight cases (EGPA: 4, CEP: 4); during the relapse treatment of one case diagnosed with EGPA, exitus occurred. One case rejected treatment despite the presence of peripheral eosinophilia, and the other cases are being followed-up without medication.
Given that the clinical pictures in pulmonary eosinophilia syndromes are on a wide spectrum, a specific diagnosis is important. Progression may differ in each patient, and a close follow-up is necessary during and after the treatment.
嗜酸性粒细胞性肺病是一组罕见的异质性疾病，其特征为气道和肺实质中嗜酸性粒细胞比例增加。在我们的诊所，对诊断为嗜酸性粒细胞性肺病的患者进行了临床特征和预后评估。
在我们的诊所，对12例诊断为嗜酸性粒细胞性肺病并进行随访的患者[嗜酸性粒细胞性肉芽肿伴多血管炎（EGPA，Churg-Strauss综合征）（n = 4）、慢性嗜酸性粒细胞性肺炎（CEP）（n = 7）和单纯性肺嗜酸性粒细胞增多症（吕弗勒综合征）（n = 1）]进行了回顾性评估。
12例患者中，8例为女性，平均年龄为43（28 - 72）岁。所有病例均因哮喘诊断接受支气管扩张剂治疗（2个月至40年）。此外，4例患者有鼻窦炎，1例有过敏性鼻炎。患者最常见的主诉是呼吸困难和咳嗽，主诉持续时间中位数为2个月。所有病例入院时均有外周血嗜酸性粒细胞增多和总IgE升高；此外，其中10例有白细胞升高，4例有贫血，4例有血小板增多。而且，记录的DLCO值中有43%低于正常。在接受支气管肺泡灌洗（BAL）的10例患者中，7例患者的嗜酸性粒细胞比例高于25%。在接受经支气管活检的8例患者中，6例检测到嗜酸性粒细胞浸润。诊断为EGPA的病例的其他发现包括鼻息肉（n = 1）、鼻窦炎（n = 2）、多发性神经病（n = 1）、心脏受累（n = 2）和活检中的皮肤受累（n = 1）。在根据患者病史、实验室检查和BAL结果进行的随访中，诊断为单纯性肺嗜酸性粒细胞增多症的患者出现自发恢复。除单纯性肺嗜酸性粒细胞增多症外，所有病例均开始使用泼尼松龙治疗并进行对照。8例患者出现复发（EGPA：4例，CEP：4例）；在1例诊断为EGPA的患者复发治疗期间，患者死亡。1例患者尽管外周血嗜酸性粒细胞增多但拒绝治疗，其他病例正在无药物治疗的情况下进行随访。
鉴于肺嗜酸性粒细胞增多综合征的临床表现范围广泛，进行特异性诊断很重要。每个患者的病情进展可能不同，治疗期间及治疗后需要密切随访。
