以弥漫性肺囊性病变伴咯血为表现的干燥综合征1例报告及文献复习

Sjgren syndrome appeared as diffuse pulmonary cystic lesion accompanied with hemoptysis:A case report and literature review

目的:探讨1例干燥综合征(SS)患者的诊疗过程并结合相关文献复习,分析SS患者的临床特征,提高临床医生对SS罕见临床症状影像学特征的认识。方法:患者,女性,23岁,因呼吸困难伴咳嗽、咳痰1个月,咯血3d就诊,查体显示睑结膜苍白,无其他明显阳性体征。胸部CT显示双肺多发囊样改变,行支气管镜活检、唇腺活检及风湿系列等其他相关检查,并给予相关治疗。结果:患者最初考虑诊断为淋巴管平滑肌瘤病(LAM)。通过相关检查最终确诊为SS,并给予口服糖皮质激素及免疫抑制剂治疗。患者经过治疗,病情好转,呼吸困难减轻,无咯血,肺弥散功能明显好转,2个月后复查患者胸部CT较治疗前无明显变化。结论:SS患者中伴有双肺多发囊样病变者罕见。SS多发生于育龄期妇女,应与LAM相鉴别。

Objective:To analyze the clinical features of Sj gren syndrome(SS)patient by investigating the diagnosis and treatment of one SS patient and perform the review of relative literatures,and to improve the understanding of the clinicians for the rare clinical symptoms and imaging features of SS.Methods:The patient was a 23-year-old woman with dyspnea associated with cough and expect oration for 1 month,and went to the hospital after hemoptysis for 3 d.The physical examination results showed pale conjunctiva and there were no other obvious positive signs.The chest CT results showed there were multiple cystic changes in both lungs.Further bronchoscopic biopsy,labial gland biopsy and rheumatism examinations and other assistant examinations were performed.The patient received related treatment.Results:The patient was initially diagnosed as lymphangioleiomyomatosis(LAM)and eventually diagnosed as SS by related examinations.The patient was treated with oral glucocorticoids and immunosuppressive agents.The symptoms of the patient were improved after treatment.The dyspnea was relieved,hemoptysis was not found,and the lung diffusion function was improved significantly.There were no significant changes in chest CT examination of the patient 2 months after treatment.Conclusion:There is rarely patients with hemoptysis and multiple cystic lesions in both lungs simultaneously among the SS patients,SS mainly occur in the women with childbearing age and it should be differentiated from LAM.