Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Department of Internal Medicine, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India.
Clin Exp Med. 2022 Nov;22(4):613-620. doi: 10.1007/s10238-021-00772-2. Epub 2021 Nov 11.
The objectives of this study were to describe the clinical features and evaluate the utility of immunological features as predictors of organ involvement and disease severity in patients with primary Sjogren's syndrome (pSS). In this single-center observational cross-sectional study, subjects fulfilling the 2012 AECG criteria or 2016 ACR/EULAR criteria for pSS were included. Details of glandular, extra-glandular manifestations, ESSDAI, ESSPRI, ANA, anti-Ro/La antibodies, rheumatoid factor (RF), complement (C3 and C4) levels and hyperglobulinemia were noted. Chi-square and Mann-Whitney U tests were performed for determining associations and relative risk (RR) was calculated. Sixty-four subjects with pSS were included in the study. Females constituted 92% and median age at onset was 37.5 (15-74) years. Ocular or oral sicca was noted in 61 (95.3%) subjects and parotidomegaly was noted in 17 (26.5%) subjects. Extra-glandular manifestations noted were: constitutional (85.9%), articular (65.6%), renal (29.6%), hematological (26.6%), cutaneous (12.5%), peripheral nerves (9.3%) and pulmonary (4.7%). Immunological features noted were: ANA (85.9%), anti-Ro (81.3%), anti-La (60.9%), RF (84.4%), hypocomplementemia (39.1%) and hyperglobulinemia (62.5%). Median ESSDAI was 6 (0-23) and ESSPRI was 7 (0-10). ANA was associated with younger age and renal involvement (RR 1.25). Anti-Ro was associated with younger age, renal involvement (RR 1.36) and high ESSDAI. Anti-La was associated with high renal (RR 3.4) and low articular involvement (RR 2.75). RF was associated with hematological involvement and hyperglobulinemia was associated with younger age. Certain immunological features can help predict the organ involvement in patients with pSS. Larger, prospective follow-up studies are needed to clearly understand these associations.
本研究的目的是描述原发性干燥综合征（pSS）患者的临床特征，并评估免疫特征作为预测器官受累和疾病严重程度的指标。在这项单中心观察性横断面研究中，纳入了符合 2012 年 AECG 标准或 2016 年 ACR/EULAR pSS 标准的患者。记录了腺体、腺体外表现、ESSDAI、ESSPRI、ANA、抗 Ro/La 抗体、类风湿因子（RF）、补体（C3 和 C4）水平和球蛋白血症的详细情况。采用卡方检验和 Mann-Whitney U 检验确定关联，计算相对风险（RR）。本研究共纳入 64 例 pSS 患者。女性占 92%，发病年龄中位数为 37.5（15-74）岁。61 例（95.3%）患者有眼干或口干，17 例（26.5%）患者有腮腺肿大。腺体外表现包括：全身症状（85.9%）、关节（65.6%）、肾脏（29.6%）、血液学（26.6%）、皮肤（12.5%）、周围神经（9.3%）和肺部（4.7%）。免疫学特征包括：ANA（85.9%）、抗 Ro（81.3%）、抗 La（60.9%）、RF（84.4%）、低补体血症（39.1%）和球蛋白血症（62.5%）。中位 ESSDAI 为 6（0-23），ESSPRI 为 7（0-10）。ANA 与年龄较小和肾脏受累相关（RR 1.25）。抗 Ro 与年龄较小、肾脏受累（RR 1.36）和高 ESSDAI 相关。抗 La 与肾脏受累高（RR 3.4）和关节受累低（RR 2.75）相关。RF 与血液学受累相关，球蛋白血症与年龄较小相关。某些免疫学特征可帮助预测 pSS 患者的器官受累。需要更大规模的前瞻性随访研究来明确这些关联。
