Department of Statistics and Operations Research, Universitat Politècnica de Catalunya, Barcelona, Spain.
Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Hungary.
Clin Exp Rheumatol. 2020 Jul-Aug;38 Suppl 126(4):85-94. Epub 2020 Oct 22.
To evaluate the systemic phenotype associated with the presence of isolated anti-La/SSB antibodies in a large international registry of patients with primary Sjögren's syndrome (pSS) fulfilling the 2002 classification criteria.
The Big Data Sjögren Project Consortium is an international, multicentre registry created in 2014. Baseline clinical information from leading centres on clinical research in SS of the 5 continents was collected. Combination patterns of anti-Ro/SSA-La/SSB antibodies at the time of diagnosis defined the following four immunological phenotypes: double positive (combined Ro/SSA and La/SSB,) isolated anti-Ro/SSA, isolated anti-La/SSB, and immunonegative.
The cohort included 12,084 patients (11,293 females, mean 52.4 years) with recorded ESSDAI scores available. Among them, 279 (2.3%) had isolated anti-La/SSB antibodies. The mean total ESSDAI score at diagnosis of patients with pSS carrying isolated anti-La/SSB was 6.0, and 80.4% of patients had systemic activity (global ESSDAI score ≥1) at diagnosis. The domains with the highest frequency of active patients were the biological (42.8%), glandular (36.8%) and articular (31.2%) domains. Patients with isolated anti-La/SSB showed a higher frequency of active patients in all ESSDAI domains but two (articular and peripheral nerve) in comparison with immune-negative patients, and even a higher absolute frequency in six clinical ESSDAI domains in comparison with patients with isolated anti-Ro/SSA. In addition, patients with isolated anti-La/SSB showed a higher frequency of active patients in two ESSDAI domains (pulmonary and glandular) with respect to the most active immunological subset (double-positive antibodies). Meanwhile, systemic activity detected in patients with isolated anti-La/SSB was overwhelmingly low. Even in ESSDAI domains where patients with isolated anti-La/SSB had the highest frequencies of systemic activity (lymphadenopathy and muscular), the percentage of patients with moderate or high activity was lower in comparison with the combined Ro/SSA and La/SSB group.
Patients carrying isolated La/SSB antibodies represent a very small subset of patients with a systemic SS phenotype characterised by a significant frequency of active patients in most clinical ESSDAI domains but with a relative low frequency of the highest severe organ-specific involvements. Primary SS still remains the best clinical diagnosis for this subset of patients.
在满足 2002 年分类标准的原发性干燥综合征（pSS）大型国际登记处中，评估存在孤立性抗 La/SSB 抗体的患者的全身表型。
大数据干燥综合征项目联盟是一个于 2014 年成立的国际多中心登记处。从 5 大洲的 SS 临床研究领先中心收集了基线临床信息。诊断时抗 Ro/SSA-La/SSB 抗体的组合模式定义了以下四种免疫表型：双阳性（联合 Ro/SSA 和 La/SSB）、孤立性抗 Ro/SSA、孤立性抗 La/SSB 和免疫阴性。
该队列纳入了 12084 名患者（11293 名女性，平均年龄 52.4 岁），记录了 ESSDAI 评分。其中，279 名（2.3%）患者存在孤立性抗 La/SSB 抗体。携带孤立性抗 La/SSB 的 pSS 患者的平均总 ESSDAI 评分在诊断时为 6.0，80.4%的患者在诊断时存在全身活动（整体 ESSDAI 评分≥1）。在所有 ESSDAI 领域中，生物（42.8%）、腺体（36.8%）和关节（31.2%）领域的活动患者比例最高。与免疫阴性患者相比，孤立性抗 La/SSB 患者在所有 ESSDAI 领域中都表现出更高的活动患者频率，甚至在与孤立性抗 Ro/SSA 患者相比的六个临床 ESSDAI 领域中，活动患者的绝对频率更高。此外，与最活跃的免疫亚群（双阳性抗体）相比，孤立性抗 La/SSB 患者在两个 ESSDAI 领域（肺部和腺体）中表现出更高的活动患者频率。同时，孤立性抗 La/SSB 患者检测到的全身活动率极高。即使在孤立性抗 La/SSB 患者具有最高全身活动频率的 ESSDAI 领域（淋巴结病和肌肉）中，中度或高度活动的患者比例也低于联合 Ro/SSA 和 La/SSB 组。
携带孤立性 La/SSB 抗体的患者代表具有全身 SS 表型的非常小的患者亚群，其特征是在大多数临床 ESSDAI 领域中具有较高频率的活动患者，但具有相对较低的最高器官特异性受累频率。原发性 SS 仍然是该患者亚群的最佳临床诊断。
