肺组织细胞增多症1例并文献复习

目的探讨肺组织细胞增多症的发病特点、诊断和治疗方法。方法结合收治的1例肺组织细胞增多症患者的临床资料，对该病的发病特点，诊断和治疗方法进行综合分析。结果肺组织细胞增多症好发于吸烟人群，临床上多表现为活动后呼吸困难以及反复气胸。X线表现为中上肺野多发小结节状改变，晚期表现为多发囊状以及纤维化改变。病理表现为嗜酸性肉芽肿。约一半病人经激素或不经任何治疗后症状缓解，三分之一病人病情急性加重，导致呼吸衰竭。结论肺组织细胞增多症是一种罕见疾病，容易误诊，HRCT对诊断很重要，确诊需要病理。治疗以激素治疗为主。

A case report of pulmonary histiocytosis with literature review

Objective To explore the characteristics of development, diagnosis and treatment of pulmonary hisfiocytesis and to improve the diagnosis ability of this rare disease. Methods One case of pulmonary histiocytosis diagnosed in our hospital was reported. The clinical data and related literatures of the disease were collected and retrospective reviewed including the clinical characteristics of its development, diagnosis and treatment methods. Results Pulmonary histiocytesis was known to occur preferentially in heavy smokers. The complains included exertional dyspnea and pneumothorax. In the chest X-ray, primarily, it involved the upper or middle lobe with micronodular. With lesion progression, cystic and fibrosis changes became prominent. Eosinophilic granuloma was the main representation in pathology. About half the patients had a favorable clinical outcome either spontaneously or with corticosteroid therapy, but approximately one third of cases showed acute deterioration and progression to respiratory in sufficiency. Conclusion Pulmonary hisfiocytosis was an uncommon disease, and can be easily misdiagnosed. HRCT has important value in diagnosing. Pathology is essential for final diagnosis. Corticesteroid therapy is the main treatment.