Sjögren Syndrome Research Group, Laboratory of Autoimmune Diseases Josep Font, IDIBAPS, Department of Autoimmune Diseases, GESCLINIC, Hospital Clínic, Barcelona, Spain.
J Rheumatol. 2010 Mar;37(3):585-90. doi: 10.3899/jrheum.090835. Epub 2010 Jan 15.
To evaluate the association between the degree of involvement shown by parotid scintigraphy at diagnosis and the disease expression, outcomes, and prognosis of primary Sjögren's syndrome (SS).
All patients consecutively diagnosed with primary SS in our department between 1984 and 2008 were evaluated. The scintigraphic stages were classified into class 4 (severe involvement), class 2-3 (mild to moderate involvement), and class 1 (normal results).
A total of 405 patients with primary SS underwent parotid scintigraphy at diagnosis (47 had class 1 involvement, 314 had class 2-3, and 44 had class 4). Patients with class 4 had a higher frequency of parotid enlargement (p < 0.001), systemic involvement (p = 0.007), high titers of antinuclear antibody (p = 0.016), positive rheumatoid factor (p = 0.002), anti-Ro/SSA (p = 0.001), anti-La/SSB (p = 0.001), low C4 levels (p = 0.001), and low CH50 (p = 0.001) in comparison with the other 2 groups. A higher rate of lymphoma development was observed in patients with class 4 involvement. Adjusted multivariate Cox regression analysis showed a hazard ratio (HR) of 10.51 (p = 0.002) and Kaplan-Meier analysis a log-rank of 0.0005. Mortality was 5-fold higher in patients with class 4 involvement. Adjusted multivariate Cox regression analysis showed an HR of 5.33 (p = 0.001) and Kaplan-Meier analysis a log-rank of 0.033.
Patients with SS presenting with severe scintigraphic involvement at diagnosis had a more pronounced autoimmune expression, a higher risk of developing systemic features and lymphoma, and a lower survival rate. Study of the degree of salivary gland dysfunction at diagnosis by parotid scintigraphy offers valuable clinical information on the prognosis and outcome of primary SS.
评估原发性干燥综合征（SS）患者在诊断时腮腺闪烁显像所显示的受累程度与疾病表现、结局和预后之间的关系。
对 1984 年至 2008 年期间在我院连续诊断为原发性 SS 的所有患者进行评估。将闪烁显像分期分为 4 级（严重受累）、2-3 级（轻度至中度受累）和 1 级（正常结果）。
共有 405 例原发性 SS 患者在诊断时进行了腮腺闪烁显像（47 例为 1 级受累，314 例为 2-3 级受累，44 例为 4 级受累）。4 级受累患者腮腺肿大的频率更高（p<0.001），全身受累的频率更高（p=0.007），抗核抗体滴度更高（p=0.016），类风湿因子阳性的频率更高（p=0.002），抗 Ro/SSA 阳性的频率更高（p=0.001），抗 La/SSB 阳性的频率更高（p=0.001），C4 水平更低（p=0.001），CH50 水平更低（p=0.001）。4 级受累患者淋巴瘤发展的发生率更高。调整后的多变量 Cox 回归分析显示，危险比（HR）为 10.51（p=0.002），Kaplan-Meier 分析对数秩为 0.0005。4 级受累患者的死亡率高 5 倍。调整后的多变量 Cox 回归分析显示，HR 为 5.33（p=0.001），Kaplan-Meier 分析对数秩为 0.033。
诊断时腮腺闪烁显像显示严重受累程度的 SS 患者具有更明显的自身免疫表达，发生全身表现和淋巴瘤的风险更高，生存率更低。通过腮腺闪烁显像评估唾液腺功能障碍程度可提供有关原发性 SS 预后和结局的有价值的临床信息。
