Department of Gastroenterology, Rheumatology and Clinical Immunology, Sapporo Medical University School of Medicine, Sapporo, Division of Advanced Medicine Promotion, Advanced Clinical Research Center, Institute of Medical Science, University of Tokyo, Tokyo, Department of Otolaryngology, Sapporo Medical University School of Medicine, Sapporo and Center for Antibody and Vaccine Therapy, Institute of Medical Science, University of Tokyo, Tokyo, Japan
Department of Gastroenterology, Rheumatology and Clinical Immunology, Sapporo Medical University School of Medicine, Sapporo, Division of Advanced Medicine Promotion, Advanced Clinical Research Center, Institute of Medical Science, University of Tokyo, Tokyo, Department of Otolaryngology, Sapporo Medical University School of Medicine, Sapporo and Center for Antibody and Vaccine Therapy, Institute of Medical Science, University of Tokyo, Tokyo, Japan.
Rheumatology (Oxford). 2015 Jan;54(1):45-9. doi: 10.1093/rheumatology/keu228. Epub 2014 Jun 6.
Inducting clinical remission by glucocorticoid treatment is relatively easy in IgG4-related disease (IgG4-RD), but relapse also occurs easily with tapering of the steroid dose. The present study tried to analyse the cases to extract predictors of relapse present at the diagnosis of IgG4-RD.
Subjects comprised 79 patients with IgG4-related dacryoadenitis and sialadenitis, known as Mikulicz's disease, who were diagnosed between April 1997 and October 2013 and followed-up for >2 years from the initial induction treatment. They were applied to Cox proportional hazard modelling, based on the outcome of interval to relapse. We performed multivariate analysis for the clinical factors of these cases and identified predictors of relapse.
Identified factors were male sex and younger onset in cases without organ involvement at diagnosis and low levels of serum IgG4 in cases with organ dysfunction at diagnosis. Complication with autoimmune pancreatitis and low steroid dose at initial treatment also tended to be associated with recurrence.
Follow-up is important in cases with recognized risk factors for relapse, including male sex and younger onset in cases without organ damage.
在 IgG4 相关疾病（IgG4-RD）中，通过糖皮质激素治疗诱导临床缓解相对容易，但随着类固醇剂量的减少，也容易复发。本研究试图分析这些病例，以提取 IgG4-RD 诊断时存在的复发预测因素。
本研究纳入了 79 例 IgG4 相关泪腺炎和涎腺炎（即 Mikulicz 病）患者，这些患者于 1997 年 4 月至 2013 年 10 月间被诊断，并在初始诱导治疗后随访超过 2 年。根据复发间隔的结果，我们对这些病例的临床因素进行了 Cox 比例风险模型分析，并确定了复发的预测因素。
未诊断为器官受累的病例中，男性和发病年龄较小，以及诊断为器官功能障碍的病例中血清 IgG4 水平较低是复发的相关因素。伴有自身免疫性胰腺炎和初始治疗时低剂量类固醇也与复发有关。
对于有复发风险因素的病例，包括无器官损害的男性和发病年龄较小的病例，随访非常重要。
