肺玻璃样变肉芽肿病一例报告并文献复习

Pulmonary hyalinizing granuloma:a case report and a review of the literature

目的 提高对罕见病肺玻璃样变肉芽肿 (PHG)的认识。方法 对 1例经尸体解剖证实的PHG患者的临床资料进行分析 ,并结合文献复习。结果 PHG是一种罕见的肺疾病 ,临床表现为咳嗽、气短、胸痛、咯血、发热、疲劳 ,若病灶融合扩大可导致呼吸衰竭 ,胸部X线片特征是双肺多发肺结节 ,显微镜下结节的特点是由涡轮状或席纹状方式排列的胶原纤维组成 ,周围有炎性细胞浸润。病因及发病机制可能与免疫有关。结论 在双肺多发肺结节的鉴别诊断中应想到PHG的可能。PHG大多预后良好 。

Objective To highlight the characteristics of pulmonary hyalinizing granuloma (PHG).Method One patient with PHG comfirmed by pathological assessment was presented and relevant literatures were reviewed.Results PHG is a rare disease characterized by multiple bilateral pulmonary nodules.Symptoms include cough,dyspnea,chest pain,hemoptysis,fever and fatigue. Histologically, the nodules consist of haphazard or whorled arrays of lamellar , keloid_like collagen.Evidence suggests that the nodules are the result of a chronic exaggerated immune response.Conclusions PHG should be considered in patients showing multiple bilateral pulmonary nodules. The clinical course of PHG in most patients is benign.At present,there is no effective therapy