Liu Xiao, Li Hao, Yin Yunhong, Ma Dedong, Qu Yiqing
Department of Respiratory Medicine, Qilu Hospital of Shandong University, Jinan, China.
Oncotarget. 2017 May 23;8(21):35473-35479. doi: 10.18632/oncotarget.16010.
Sjögren's syndrome (SS) is a chronic inflammatory autoimmune disease that can occur as a unique existence (primary Sjögren's syndrome) or merge with other systemic diseases like systemic lupus erythematosus (SLE), rheumatoid arthritis or systemic sclerosis (secondary Sjögren's syndrome). Data on the two diseases occurrence order are inadequate. Primary Sjögren's syndrome (pSS) may relatively uncommonly lead to diffuse cystic lung changes. We represent a female who was diagnosed pSS with diffuse cystic lung alterations developed SLE two years later. SS was diagnosed on account of the existence of dryness of eye and mouth, Schirmer's test, biopsy of the minor salivary glands of her lip, positive anti-SSA and anti-SSB antibody in the serum. Chest computed tomography image showed bilateral diffuse cystic changes with a wide variation in cyst size and distribution. SLE was finally diagnosed based on bilateral lower limb skin rash, gonarthritis and omarthritis, low level of complement, antinuclear antibody 1:640 and positive antibodies to double-stranded DNA. Improvement was achieved with therapy of corticosteroids, hydroxychloroquine and antibiotics. This report provides us clinical, diagnosis and treatment perception of SS-onset SLE as patient presenting diffuse cystic lung changes.
干燥综合征（SS）是一种慢性炎症性自身免疫性疾病，可单独出现（原发性干燥综合征），也可与其他系统性疾病合并，如系统性红斑狼疮（SLE）、类风湿关节炎或系统性硬化症（继发性干燥综合征）。关于这两种疾病发病顺序的数据不足。原发性干燥综合征（pSS）相对较少导致弥漫性囊性肺改变。我们报告一例女性患者，诊断为pSS伴弥漫性囊性肺改变，两年后发展为SLE。根据眼干、口干、泪液分泌试验、唇部小唾液腺活检、血清中抗SSA和抗SSB抗体阳性诊断为SS。胸部计算机断层扫描图像显示双侧弥漫性囊性改变，囊肿大小和分布差异很大。最终根据双侧下肢皮疹、膝关节炎和肩关节炎、补体水平降低、抗核抗体1:640及双链DNA抗体阳性诊断为SLE。通过皮质类固醇、羟氯喹和抗生素治疗病情得到改善。本报告为我们提供了pSS合并SLE，伴有弥漫性囊性肺改变患者的临床、诊断和治疗经验。
