Rheumatol Int. 2010 Feb;30(4):565-8. doi: 10.1007/s00296-009-1030-4. Epub 2009 Jul 17.
It is considered that autoimmune pancreatitis (AIP), Mikulicz's disease (MD) and IgG4-related tubulointerstitial nephritis (TIN) comprise systemic IgG4-related plasmacytic syndrome (SIPS), of which the origin remains unknown. We analyzed these patients with focus on serological aspects to invest whether there are autoantigens in SIPS. We evaluated 28 patients with SIPS who presented at Sapporo Medical University Hospital and the collaborated institutions. They were mainly middle-aged (eight male), and consisted of 26 patients with MD and two patients with AIP. The three among 26 patients diagnosed with MD were complicated to AIP, and another three patients had IgG4-related TIN. As a control, healthy volunteers and the patients with Sjögren's syndrome were examined. At first, we measured the levels of serum complements and circulating immune complexes in these patients. Next, immune complexes were collected from the serum of patients and healthy controls by immunoprecipitation. They were divided into immunoglobulin and the antigens by glycine-HCl solution. The divided samples including the antigens were analyzed by surface-enhanced laser desorption/ionization-time of flight-mass spectrometry (SELDI-TOF-MS). Nine patients had hypocomplementemia and 15 had elevated levels of circulating immune complexes in the group of SIPS. In the groups of healthy volunteers and SS, all showed that the levels of serum complements and circulating immune complexes were normal. SELDI-TOF-MS detected a 13.1-kDa protein from all samples of SIPS, and not in normal control and SS. It is possible that the 13.1-kDa protein is one of the autoantigens of SIPS.
人们认为自身免疫性胰腺炎（AIP）、Mikulicz 病（MD）和 IgG4 相关的肾小管间质性肾炎（TIN）组成系统性 IgG4 相关浆细胞综合征（SIPS），其病因尚不清楚。我们分析了这些患者，重点关注血清学方面，以研究 SIPS 是否存在自身抗原。我们评估了在札幌医科大学医院和合作机构就诊的 28 例 SIPS 患者。他们主要为中年人（8 名男性），包括 26 例 MD 患者和 2 例 AIP 患者。26 例 MD 患者中有 3 例并发 AIP，另有 3 例患者合并 IgG4 相关 TIN。作为对照，检查了健康志愿者和干燥综合征患者。首先，我们测量了这些患者血清补体和循环免疫复合物的水平。然后，通过免疫沉淀从患者和健康对照者的血清中收集免疫复合物。用甘氨酸-HCl 溶液将免疫复合物分为免疫球蛋白和抗原。通过表面增强激光解吸/电离飞行时间质谱（SELDI-TOF-MS）分析分离的样品中的抗原。9 例 SIPS 患者存在低补体血症，15 例患者存在循环免疫复合物水平升高。在健康志愿者和 SS 组中，所有患者的血清补体和循环免疫复合物水平均正常。SELDI-TOF-MS 从所有 SIPS 样本中检测到一种 13.1kDa 的蛋白质，而在正常对照组和 SS 中未检测到。该 13.1kDa 蛋白可能是 SIPS 的自身抗原之一。
