Ter Borg E J, Kelder J C
Department of Rheumatology, St. Antonius Hospital, Koekoekslaan 1, 3435 CM, Nieuwegein, The Netherlands.
Department of Clinical Epidemiology and Medical Statistics, St. Antonius Hospital, Koekoekslaan 1, 3435 CM, Nieuwegein, The Netherlands.
Rheumatol Int. 2017 Jul;37(7):1153-1158. doi: 10.1007/s00296-017-3715-4. Epub 2017 Apr 19.
To investigate in a long-term study, the development of new extra-glandular manifestations (EGM) or associated auto-immune diseases (AID) from 1 year after establishing the diagnosis of primary Sjögren's syndrome (pSS). The primary goal was to examine the frequency and type of these manifestations and to find out which demographic, clinical and serological profile was most at risk. All outpatients diagnosed with primary Sjögren's syndrome were included in a retrospective study, with at least one check-up per year, from June 1991 until August 2015. Patients also fulfilling the criteria for concomitant connective tissue disorders were excluded. Data were collected with respect to the cumulative prevalence of a new EGM or associated AID. 140 patients were included in the final analysis. After 10 years of follow-up, the cumulative incidence of a new EGM or associated AID was 30.7%. The most frequent events were polyneuropathy, interstitial lung disease, (poly)arthritis, discoid lupus erythematosus (LE)/subacute cutaneous LE and Hashimoto's disease. Non-Hodgkin lymphoma was not diagnosed during the follow-up. Patients without chronic benign pain syndrome (CBP) (HR 2.13; 95% CI [0.94-4.76]; p = 0.061), but in particular those with cryoglobulins (HR 2.87; 95% CI [1.20-6.86]; p = 0.013), developed more events. Age at diagnosis, gender, the presence of ANA, anti-Ro/SSA, anti-La/SSB, IgM-RF, decreased levels of C3 or C4, or hypergammaglobulinaemia did not show any statistically significant differences. The burden of disease in pSS is higher than expected due to the development of EGM or associated AID. Therefore, we recommend long-term follow-up of all pSS patients, particularly those with cryoglobulinaemia.
在一项长期研究中，调查原发性干燥综合征（pSS）确诊1年后新的腺外表现（EGM）或相关自身免疫性疾病（AID）的发生情况。主要目标是检查这些表现的频率和类型，并找出哪些人口统计学、临床和血清学特征最具风险。所有诊断为原发性干燥综合征的门诊患者纳入一项回顾性研究，研究时间为1991年6月至2015年8月，每年至少进行一次检查。同时符合结缔组织病标准的患者被排除。收集有关新的EGM或相关AID累积患病率的数据。最终分析纳入了140例患者。随访10年后，新的EGM或相关AID的累积发病率为30.7%。最常见的事件是多发性神经病、间质性肺病、（多）关节炎、盘状红斑狼疮（LE）/亚急性皮肤型LE和桥本氏病。随访期间未诊断出非霍奇金淋巴瘤。无慢性良性疼痛综合征（CBP）的患者（风险比[HR]2.13；95%置信区间[CI][0.94 - 4.76]；p = 0.061），尤其是有冷球蛋白的患者（HR 2.87；95% CI[1.20 - 6.86]；p = 0.013），发生更多此类事件。诊断时的年龄、性别、抗核抗体（ANA）、抗Ro/SSA、抗La/SSB、IgM类风湿因子（RF）、C3或C4水平降低或高球蛋白血症均未显示出任何统计学上的显著差异。由于EGM或相关AID的发生，pSS的疾病负担高于预期。因此，我们建议对所有pSS患者进行长期随访，尤其是冷球蛋白血症患者。
