Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Department of Pathology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Mod Rheumatol. 2020 Jan;30(1):172-177. doi: 10.1080/14397595.2019.1572575. Epub 2019 Feb 18.
To evaluate if major salivary gland enlargement in patients with IgG4-related disease (IgG4-RD) is associated with systemic involvement. We included 47 patients with diagnosis of IgG4-RD. We retrospectively collected demographics, organ involvement, disease activity and damage assessed by the IgG4-RD Responder Index (IgG4-RD RI) and treatment. 25 patients (53%) were men, mean age 50.8 years and median disease duration 27 months. Most frequently anatomic sites affected were lymph nodes 55%, pancreas 51% and lacrimal glands 43%. We observed major salivary gland involvement in 22 (46.8%) patients. When we compared patients with ( = 22) vs. without ( = 25) salivary gland enlargement, the first group had a higher number of affected organs, a higher prevalence of lacrimal glands, lymph nodes, and lung involvement, rheumatoid factor positivity, azathioprine and prednisone use, as well as a higher baseline IgG4-RD RI and a longer delay in diagnosis. At logistic regression analysis we found an association of major salivary gland enlargement with the basal IgG4-RD RI (OR 1.37, 95%CI 1.09-1.61, = .001) and with lacrimal gland involvement (OR 34.7, 95%CI 4.6-258, = .001). Our study highlights the systemic nature of IgG4-RD. Patients with major salivary gland enlargement should be routinely screened for multi-organ disease.
为了评估 IgG4 相关疾病（IgG4-RD）患者的主要唾液腺肿大是否与全身受累有关。我们纳入了 47 例 IgG4-RD 患者。我们回顾性收集了人口统计学、器官受累、疾病活动度和通过 IgG4-RD 应答指数（IgG4-RD RI）评估的损害以及治疗情况。25 例（53%）为男性，平均年龄 50.8 岁，中位疾病病程 27 个月。最常受累的解剖部位为淋巴结 55%、胰腺 51%和泪腺 43%。我们观察到 22 例（46.8%）患者存在主要唾液腺受累。当我们比较有（ = 22）和无（ = 25）唾液腺肿大的患者时，前者受累器官更多，泪腺、淋巴结和肺部受累的发生率更高，类风湿因子阳性，使用硫唑嘌呤和泼尼松，以及更高的基线 IgG4-RD RI 和更长的诊断延迟。在逻辑回归分析中，我们发现主要唾液腺肿大与基础 IgG4-RD RI（OR 1.37，95%CI 1.09-1.61， = .001）和泪腺受累（OR 34.7，95%CI 4.6-258， = .001）相关。我们的研究强调了 IgG4-RD 的全身性。有主要唾液腺肿大的患者应常规筛查多器官疾病。
