Division of Rheumatology, Department of Internal Medicine, Soonchunhyang University Seoul Hospital, Soonchunhyang University School of Medicine, Daesagwan-ro, Yongsan-gu, 04401 59 Seoul, Republic of Korea.
Division of Rheumatology, Department of Internal Medicine, Konkuk University Medical Center, Konkuk University School of Medicine, Seoul, South Korea.
Joint Bone Spine. 2021 Jul;88(4):105132. doi: 10.1016/j.jbspin.2021.105132. Epub 2021 Jan 21.
To assess the clinical, laboratory, and salivary gland ultrasound (SGUS) characteristics of elderly-onset of primary Sjögren's syndrome (EopSS).
We included pSS patient from two referral hospitals over a 4-year period. The SGUS scores (0-48) and SG volumes were assessed. Clinical, laboratory, and SGUS data were compared according to age at onset: EopSS (≥65 years), adult-onset (AopSS) (≥40 and <65 years), and young-onset (YopSS) (<40 years).
This cross-sectional study included a total of 221 patients, 43 (19.5%) of which had EopSS. Subjective sicca symptoms, results of the Schirmer's test, and unstimulated salivary flow rate revealed no significant differences between the groups. EopSS patients presented a significantly higher frequency of interstitial lung disease (ILD) (EopSS: 51.2% vs. AopSS: 13.5% vs. YopSS: 8.7%, P<0.001) and lower frequency of arthritis (7% vs. 22.6% vs. 39.1%, P<0.01). They also had significantly lower positivity of anti-Ro/SSA (51.2%) and anti-La/SSB (7.0%) and lower levels of rheumatoid factor, C4, and IgG. The EopSS group had significantly lower SGUS positivity (defined as total scores ≥14) (44.2% vs. 64.5% vs. 78.3%, P<0.05), lower SGUS scores, and smaller submandibular gland volume.
We report a specific phenotype of EopSS, characterised by high prevalence of ILD, less involvement of the peripheral joint, and low biological activity. SGUS evaluation showed less parenchymal abnormalities but more atrophic changes in major SGs in EopSS patients. Considering the low positivity of anti-Ro/SSA and SGUS in EopSS, SG biopsy remains the only way to confirm the diagnosis of pSS, especially in elderly patients.
评估老年原发性干燥综合征（EopSS）的临床、实验室和唾液腺超声（SGUS）特征。
我们纳入了 4 年来在两家转诊医院就诊的 pSS 患者。评估了 SGUS 评分（0-48）和 SG 体积。根据发病年龄比较了临床、实验室和 SGUS 数据：EopSS（≥65 岁）、成人发病（AopSS）（≥40 岁且<65 岁）和青少年发病（YopSS）（<40 岁）。
这项横断面研究共纳入了 221 例患者，其中 43 例（19.5%）为 EopSS。主观干燥症状、Schirmer 试验结果和非刺激性唾液流量在各组之间无显著差异。EopSS 患者发生间质性肺病（ILD）的频率明显更高（EopSS：51.2% vs. AopSS：13.5% vs. YopSS：8.7%，P<0.001），关节炎频率更低（7% vs. 22.6% vs. 39.1%，P<0.01）。EopSS 组抗 Ro/SSA 的阳性率也明显更低（51.2%）和抗 La/SSB 更低（7.0%），类风湿因子、C4 和 IgG 水平也更低。EopSS 组 SGUS 阳性率（定义为总分≥14）明显更低（44.2% vs. 64.5% vs. 78.3%，P<0.05），SGUS 评分和颌下腺体积也更小。
我们报告了 EopSS 的一种特定表型，其特点是ILD 患病率高、外周关节受累少且生物学活性低。SGUS 评估显示 EopSS 患者的实质异常较少，但主要 SG 的萎缩性改变更多。鉴于 EopSS 患者抗 Ro/SSA 和 SGUS 的阳性率较低，SG 活检仍然是诊断 pSS 的唯一方法，尤其是在老年患者中。
