Gao Hui, Zou Ya-Dan, Zhang Xue-Wu, He Jing, Zhang Jing, Sun Ye, Li Zhan-Guo
Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing, China.
Department of Rheumatology and Immunology, Peking University International Hospital, Beijing, China.
Int J Rheum Dis. 2018 Jul;21(7):1423-1429. doi: 10.1111/1756-185X.13334.
This study investigated the common initial clinical presentations of primary Sjögren's syndrome (pSS) with interstitial lung disease (ILD) and explored differences between sicca and non-sicca onset pSS-ILD patients.
A total of 1341 SS patients hospitalized between 2003 and 2012 were retrospectively reviewed. Of them, 102 were analyzed and recruited to examine the differences between non-sicca and sicca onset.
Fifty-one percent of pSS-ILD patients presented with non-sicca onset. Although the mean diagnosis time was equal, only 4% of non-sicca onset patients were diagnosed with pSS at onset versus 34% with sicca onset (P = 0.000). Hyperglobulinemia, elevated rheumatoid factor (RF) titer, and anti-SSA and/or anti-SSB presence were less predominant in patients with non-sicca onset (immunoglobulin G, 16 [12-21] vs. 21 [15-28] g/L, P = 0.032; RF, 22 [20-171] vs. 104 [20-237] IU/mL, P = 0.048; anti-SSA and/or anti-SSB presence, 33% vs. 72%, P = 0.000). The usual interstitial pneumonia pattern was more commonly seen in non-sicca onset patients (20.0% vs. 14.3%). The high-resolution computed tomography score was higher (12 [88-15] vs. 8 [5-13], P = 0.070) and predicted total lung capacity and forced vital capacity were lower (87 ± 23% vs. 97 ± 20%, P = 0.050; 88 ± 28% vs. 100 ± 27%, P = 0.089) in non-sicca patients.
Non-sicca is a common initial manifestation in pSS-ILD. Anti-SSA presence, elevated RF titer and hyperglobulinemia were less predominant, and pulmonary complications were more progressive and severe in non-sicca onset patients than sicca onset patients.
本研究调查了原发性干燥综合征（pSS）合并间质性肺疾病（ILD）的常见初始临床表现，并探讨了干燥型和非干燥型起病的pSS-ILD患者之间的差异。
回顾性分析2003年至2012年期间住院的1341例SS患者。其中，102例被分析并纳入研究，以检查非干燥型和干燥型起病之间的差异。
51%的pSS-ILD患者为非干燥型起病。尽管平均诊断时间相同，但非干燥型起病患者中仅有4%在起病时被诊断为pSS，而干燥型起病患者为34%（P = 0.000）。非干燥型起病患者中高球蛋白血症、类风湿因子（RF）滴度升高以及抗SSA和/或抗SSB的存在较少见（免疫球蛋白G，16[12 - 21] vs. 21[15 - 28]g/L，P = 0.032；RF，22[20 - 171] vs. 104[20 - 237]IU/mL，P = 0.048；抗SSA和/或抗SSB的存在，33% vs. 72%，P = 0.000）。非干燥型起病患者更常见普通型间质性肺炎模式（20.0% vs. 14.3%）。非干燥型患者的高分辨率计算机断层扫描评分更高（12[8 - 15] vs. 8[5 - 13]，P = 0.070），预计总肺活量和用力肺活量更低（87±23% vs. 97±20%，P = 0.050；88±28% vs. 100±27%，P = 0.089）。
非干燥型是pSS-ILD的常见初始表现。非干燥型起病患者中抗SSA的存在、RF滴度升高和高球蛋白血症较少见，且肺部并发症比干燥型起病患者更进展和严重。
