Marchand E, Reynaud-Gaubert M, Lauque D, Durieu J, Tonnel A B, Cordier J F
Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires, Hôpital Cardiovasculaire et Pneumologique Louis Pradel, Lyon, France.
Medicine (Baltimore). 1998 Sep;77(5):299-312. doi: 10.1097/00005792-199809000-00001.
Idiopathic chronic eosinophilic pneumonia (CEP) is a rare disorder of unknown cause with nonspecific respiratory and systemic symptoms but rather characteristic peripheral alveolar infiltrates on imaging, developing mainly in women and in atopic subjects. The disorder is highly responsive to oral corticosteroid therapy, but relapses are frequent on reducing or stopping treatment. The long-term course of the disease and data regarding outcome, particularly the need for prolonged oral corticosteroid therapy and the development of severe asthma, are somewhat contradictory. A multicentric retrospective study was conducted in an attempt to describe better the initial features and, above all, the later course of CEP in a large homogeneous series of 62 stringently selected patients of whom 46 were followed for more than 1 year. The prevalence of smokers was low (6.5%) and about half of our patients (51.6%) had a previous, and often prolonged, history of asthma. The clinical and roentgenographic features were in keeping with previous studies, but we found that computed tomography could disclose ground glass opacities not detected by X-ray, and that migratory infiltrates before treatment were more frequent (25.5%) than reported previously. The bronchoalveolar lavage cellular count always showed a striking eosinophilic pattern, thus allowing distinction between CEP and cryptogenic organizing pneumonia, both syndromes sharing many common clinical and imaging features. About two-thirds of the patients (68%) showed a ventilatory defect in pulmonary function tests, with about one-half of these presenting with an obstructive pattern, sometimes without previous asthma. Along with the submucosal eosinophilic infiltration noted in 2 patients without ventilatory defect, this is strong evidence to confirm that CEP is not only an alveolointerstitial but also an airway disease. The dramatic response to oral corticosteroid therapy was observed in all treated patients. Although only 1 patient initially treated for less than 6 months did not relapse, longer oral corticosteroid therapy in no way provided protection from further relapses. We thus propose to try to wean oral corticosteroid therapy after 6 months in patients without severe asthma, because recurrences remain responsive to oral steroids. However, prolonged oral corticosteroid therapy was necessary in the majority of patients, with 68.9% of those followed for more than 1 year still on oral corticosteroid therapy at the last follow-up, either because of relapse or because of severe asthma.
特发性慢性嗜酸性粒细胞性肺炎（CEP）是一种病因不明的罕见疾病，具有非特异性的呼吸道和全身症状，但影像学上有特征性的外周肺泡浸润，主要发生在女性和特应性个体中。该疾病对口服糖皮质激素治疗反应良好，但在减少或停止治疗后复发频繁。疾病的长期病程及预后数据，尤其是长期口服糖皮质激素治疗的必要性和严重哮喘的发生情况，存在一定矛盾。我们进行了一项多中心回顾性研究，旨在更好地描述62例经过严格筛选的患者的初始特征，尤其是CEP的后期病程，其中46例患者随访时间超过1年。吸烟者的比例较低（6.5%），约一半的患者（51.6%）既往有哮喘病史，且往往病程较长。临床和影像学特征与先前的研究一致，但我们发现计算机断层扫描能够发现X线未检测到的磨玻璃影，且治疗前的游走性浸润更为常见（25.5%），高于先前报道。支气管肺泡灌洗细胞计数始终显示出明显的嗜酸性粒细胞模式，从而能够区分CEP和隐源性机化性肺炎，这两种综合征具有许多共同的临床和影像学特征。约三分之二的患者（68%）在肺功能测试中显示通气功能障碍，其中约一半表现为阻塞性模式，有些患者既往并无哮喘病史。在2例无通气功能障碍的患者中观察到黏膜下嗜酸性粒细胞浸润，这有力地证明CEP不仅是一种肺泡间质疾病，也是一种气道疾病。所有接受治疗的患者对口服糖皮质激素治疗均有显著反应。尽管最初治疗时间不足6个月的患者中只有1例未复发，但更长时间的口服糖皮质激素治疗并不能防止进一步复发。因此，我们建议对于无严重哮喘的患者，在6个月后尝试停用口服糖皮质激素治疗，因为复发对口服类固醇药物仍有反应。然而，大多数患者需要长期口服糖皮质激素治疗，在最后一次随访时，随访时间超过1年的患者中有68.9%仍在接受口服糖皮质激素治疗，原因要么是复发，要么是患有严重哮喘。
