Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.
Int J Rheum Dis. 2019 May;22(5):929-933. doi: 10.1111/1756-185X.13464. Epub 2018 Dec 26.
AIM/INTRODUCTION: Primary Sjögren's syndrome (pSS) is a prototypical systemic autoimmune disease that manifests with various signs and symptoms. Although some studies have examined these manifestations over the long-term course of the disease, the association between initial clinical and immunological factors and subsequent long-term manifestations has not been fully elucidated. The aim of this study is to identify initial clinical and immunological factors associated with subsequent manifestations in patients with pSS.
A retrospective review was performed in pSS patients who were followed up over a 10-year period in our department. Clinical and immunological data, including serum immunoglobulin (Ig) and autoantibody levels, were collected and statistically analyzed.
We analyzed 91 patients who were followed up in our department. The proportion of patients with extraglandular involvement decreased from 90% to 73%, while eight patients developed extraglandular organ involvement. Extraglandular involvement at 10 years more frequently occurred in patients with hyper-IgG than those without hyper-IgG at initial testing (P < 0.01). Extraglandular organ involvement at 10 years more frequently occurred in rheumatoid factor (RF)-positive patients at the time of SS diagnosis (P < 0.05). Malignancy occurred in 9% of patients. Age, lower CH50 and thrombocytopenia were significantly associated with malignancy. Extraglandular organ involvement was associated with the presence of hyper-IgG and RF positivity (P < 0.01 and P < 0.05).
Our study identified important initial clinical and immunological factors associated with subsequent manifestations in patients with pSS over a long follow-up period. pSS patients with RF and hyper-IgG at diagnosis may have a higher risk of subsequent extraglandular involvement.
目的/引言：原发性干燥综合征（pSS）是一种典型的系统性自身免疫性疾病，表现为各种症状和体征。尽管一些研究已经在疾病的长期过程中检查了这些表现，但初始临床和免疫学因素与随后的长期表现之间的关系尚未完全阐明。本研究旨在确定与 pSS 患者随后表现相关的初始临床和免疫学因素。
对我科随访 10 年的 pSS 患者进行回顾性分析。收集并统计分析临床和免疫学数据，包括血清免疫球蛋白（Ig）和自身抗体水平。
我们分析了 91 例在我科随访的患者。有外分泌腺受累的患者比例从 90%下降至 73%，而 8 例患者出现了外分泌腺外器官受累。初诊时 IgG 升高的患者在 10 年后发生外分泌腺外器官受累的比例高于 IgG 正常的患者（P<0.01）。在 SS 诊断时 RF 阳性的患者在 10 年后更易发生外分泌腺外器官受累（P<0.05）。9%的患者发生了恶性肿瘤。年龄、较低的 CH50 和血小板减少与恶性肿瘤显著相关。外分泌腺外器官受累与 IgG 升高和 RF 阳性相关（P<0.01 和 P<0.05）。
本研究在长期随访中确定了与 pSS 患者随后表现相关的重要初始临床和免疫学因素。初诊时 RF 和 IgG 升高的 pSS 患者可能有更高的发生外分泌腺外器官受累的风险。
