First Department of Internal Medicine, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan.
Rheumatology (Oxford). 2013 Apr;52(4):679-83. doi: 10.1093/rheumatology/kes358. Epub 2012 Dec 19.
Despite ongoing research, the clinical and histopathological natural history of immunoglobulin (Ig) G4-related disease (IgG4-RD) remains unclear and the optimal time to initiate treatment is unknown. A focus on clinical symptoms rather than image finding is recommended for therapeutic initiation in autoimmune pancreatitis, but evidence for this approach is lacking. We aimed to retrospectively analyse disease duration, efficacy of treatment with glucocorticoids and results of histopathological examination of submandibular gland specimens to clarify the necessity for early intervention in IgG4-RD.
Salivary secretions were assessed before and after treatment in 26 cases of IgG4-related Mikulicz's disease (IgG4-MD). Relationships between disease duration, amount of salivary secretion before treatment, improvement of salivary secretion and ratios of areas of residual acini, fibrosis and lymphoid follicles in the involved submandibular gland specimens were analysed.
Salivary secretions were significantly reduced in cases with illness of >2 years (P < 0.05). An inverse correlation was seen between improved amount of salivary secretion and amount of salivary secretion before treatment (r = -0.60). Improved amount of salivary secretion was also associated with each histological factor (acini, r = 0.29; fibrosis, r = -0.23; lymphoid follicles, r = -0.31), which showed interrelationships (acini and lymphoid follicles, r = -0.23; acini and fibrosis, r = 0.42; lymphoid follicles and fibrosis, r = 0.30).
Salivary secretion can be improved even in cases with lower levels of salivary secretion before treatment in IgG4-RD, but improvements in the amount of salivary secretion decrease with histological changes with delayed therapeutic intervention. These data suggest that early intervention is needed to improve outcomes in patients with IgG4-MD.
尽管不断有研究，但免疫球蛋白（Ig）G4 相关疾病（IgG4-RD）的临床和组织病理学自然史仍不清楚，也不清楚开始治疗的最佳时间。推荐在自身免疫性胰腺炎中根据临床症状而不是影像学发现来启动治疗，但缺乏这种方法的证据。我们旨在回顾性分析疾病持续时间、糖皮质激素治疗的疗效以及颌下腺标本的组织病理学检查结果，以阐明 IgG4-RD 早期干预的必要性。
对 26 例 IgG4 相关 Mikulicz 病（IgG4-MD）患者的唾液分泌进行了治疗前后的评估。分析了疾病持续时间、治疗前唾液分泌量、唾液分泌改善情况以及颌下腺受累标本中残余腺泡、纤维化和淋巴滤泡面积比之间的关系。
患病 2 年以上的病例唾液分泌明显减少（P < 0.05）。唾液分泌量改善与治疗前唾液分泌量呈负相关（r = -0.60）。唾液分泌量的改善也与每种组织学因素相关（腺泡，r = 0.29；纤维化，r = -0.23；淋巴滤泡，r = -0.31），这些因素之间存在相互关系（腺泡和淋巴滤泡，r = -0.23；腺泡和纤维化，r = 0.42；淋巴滤泡和纤维化，r = 0.30）。
即使在 IgG4-RD 治疗前唾液分泌水平较低的情况下，也可以改善唾液分泌，但随着治疗干预的延迟，唾液分泌量的改善会减少，组织学变化也会减少。这些数据表明，需要早期干预以改善 IgG4-MD 患者的预后。
