关键词: 干燥综合征, 淋巴管平滑肌瘤病, 弥漫性肺囊样病变, 咯血, 病例报告

Abstract: 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 expectoration 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.

Key words: Sjögren syndrome, lymphangioleiomyomatosis, diffuse pulmonary cystic lesion, hemostasis, case report