Department of Rheumatology, Faculty of Medicine, Ankara University, Ankara, Turkiye.
Department of Hematology, Faculty of Medicine, Ankara University, Ankara, Turkiye.
Turk J Med Sci. 2024 Oct 7;54(5):956-962. doi: 10.55730/1300-0144.5873. eCollection 2024.
BACKGROUND/AIM: The objective of this study was to demonstrate the commonalities and distinctions between patients with seronegative and seropositive primary Sjögren's syndrome (pSS).
The records of 399 patients with pSS seen between January 2010 and June 2023 were retrospectively examined. Patients with negative antiSSA/Ro, antiSSB/La, ANA, and RF antibodies comprised the seronegative group, while patients with at least one positive antibody were included in the seropositive group.
The most common clinical features between the groups were arthralgia (81.2%), arthritis (11.5%), hematological involvement (19.8%), and pulmonary involvement (11.8%). In 41 patients (10.3%), no autoantibody positivity was detected. The number of patients with at least one extraarticular involvement was statistically more frequent in the seropositive group (p = 0.011). Dry mouth was found to be more prevalent among seronegative patients (p = 0.003). While hyperimmune gammaglobulinemia exhibited a higher prevalence within the seropositive group (p = 0.004), the occurrence of reduced complement levels was at similar rates in both groups. All deaths were observed exclusively within the seropositive group (17/358, 4.7%). No difference was observed between the two groups concerning mortality (p = 0.237) and malignancies (seropositive group: 9/358, 2.5% vs. seronegative group: 3/41, 7.3%, p = 0.115). There was a statistically significant association between low C4 levels (OR = 2.99 [1.09-8.16], p = 0.045 in model 1, OR = 3.10 [1.14-8.42], p = 0.022 in model 2), and the extraarticular findings.
While hematological, renal, pulmonary, and neurological involvements are observed with similar frequency in both seronegative and seropositive pSS patients, the presence of extraarticular manifestations was more common in seropositive patients. Additionally, there was a relationship between extraarticular involvement and low C4 levels.
背景/目的：本研究旨在展示血清阴性和血清阳性原发性干燥综合征（pSS）患者之间的共性和差异。
回顾性分析了 2010 年 1 月至 2023 年 6 月期间就诊的 399 例 pSS 患者的病历。抗 SSA/Ro、抗 SSB/La、ANA 和 RF 抗体阴性的患者组成血清阴性组，至少有一种抗体阳性的患者归入血清阳性组。
两组最常见的临床特征是关节痛（81.2%）、关节炎（11.5%）、血液学受累（19.8%）和肺部受累（11.8%）。在 41 例（10.3%）患者中，未检测到任何自身抗体阳性。血清阳性组至少有一种关节外受累的患者数量统计学上更为频繁（p = 0.011）。血清阴性组患者的口干更为常见（p = 0.003）。高免疫球蛋白血症在血清阳性组中更为常见（p = 0.004），而两组补体水平降低的发生率相似。所有死亡均发生在血清阳性组（358 例中的 17 例，4.7%）。两组之间的死亡率（血清阳性组：17/358，4.7% vs. 血清阴性组：3/41，7.3%，p = 0.237）和恶性肿瘤（血清阳性组：9/358，2.5% vs. 血清阴性组：3/41，7.3%，p = 0.115）均无差异。低 C4 水平（模型 1 中 OR = 2.99[1.09-8.16]，p = 0.045，模型 2 中 OR = 3.10[1.14-8.42]，p = 0.022）与关节外表现之间存在统计学显著关联。
虽然血清阴性和血清阳性 pSS 患者的血液学、肾脏、肺部和神经系统受累发生率相似，但血清阳性患者的关节外表现更为常见。此外，关节外受累与低 C4 水平之间存在关系。
