Development of IgG4-related pancreatitis and kidney disease 7?years after the onset of undiagnosed lymphadenopathy: A case report
Development of IgG4-related pancreatitis and kidney disease 7?years after the onset of undiagnosed lymphadenopathy: A case report
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未确诊淋巴结肿大发病 7 年后发生 IgG4 相关胰腺炎和肾脏疾病：病例报告
DOI：
10.1093/mrcr/rxac065
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发表时间：
2022
影响因子：
0.8
通讯作者：
Kawano Mitsuhiro
中科院分区：
文献类型：
--
作者：
Yoshida Misaki;Mizushima Ichiro;Tsuge Shunsuke;Takahashi Yoshinori;Zoshima Takeshi;Nishioka Ryo;Hara Satoshi;Ito Kiyoaki;Kawano Mitsuhiro
本报告描述了一个病人被诊断为免疫球蛋白G4（IgG 4）相关的胰腺炎和肾脏疾病7年后发病的未确诊的淋巴结病。一名48岁的日本女性，因疲劳和腿部水肿就诊。计算机断层扫描显示胃周淋巴结病，为此她接受了腹腔镜胃周淋巴结活检。尽管淋巴结的组织病理学检查未导致明确诊断，但血清学试验显示血清IgG 4水平升高（558 mg/dl），淋巴结的IgG 4免疫染色显示IgG 4阳性浆细胞浸润，导致疑似IgG 4相关疾病。进一步检查显示，除淋巴结病外，无其他器官病变。在55岁时，尽管没有主观症状，对比增强计算机断层扫描显示胰腺尾部和左肾低密度病变。组织学检查显示两个器官中淋巴细胞浸润（由浆细胞和嗜酸性粒细胞混合物组成）和胰腺闭塞性静脉炎。肾脏标本的IgG 4免疫染色显示每个高倍视野有160个IgG 4阳性细胞，IgG 4 +/IgG+细胞比率几乎为100%，从而诊断为IgG 4相关胰腺炎和肾脏疾病。泼尼松龙治疗2个月后，病变改善。尽管IgG 4相关淋巴结病的诊断在单独淋巴结病患者中通常具有挑战性，但本患者的研究结果强调了长期随访的重要性，因为它可以早期检测IgG 4相关疾病累及其他器官。
This report describes a patient diagnosed with immunoglobulin G4 (IgG4)-related pancreatitis and kidney disease 7 years after the onset of undiagnosed lymphadenopathy. A 48-year-old Japanese woman presented with fatigue and leg oedema. Computed tomography showed perigastric lymphadenopathy, for which she underwent a laparoscopic biopsy of the perigastric lymph nodes. Although histopathological examination of the lymph nodes did not lead to a definitive diagnosis, serological tests revealed elevated serum IgG4 levels (558 mg/dl) and IgG4 immunostaining of the lymph nodes showed IgG4-positive plasma cell infiltration, leading to the suspicion of IgG4-related disease. Further workup revealed no organ lesion other than lymphadenopathy. At age 55 years, despite having no subjective symptoms, contrast-enhanced computed tomography showed low-density lesions in the tail of the pancreas and the left kidney. Histopathological examination showed lymphocyte infiltration, consisting of a mixture of plasma cells and eosinophils, in both organs and obliterative phlebitis in the pancreas. IgG4 immunostaining of the kidney specimens showed 160 IgG4-positive cells per high-powered field, with the IgG4+/IgG+ cell ratio being almost 100%, leading to a diagnosis of IgG4-related pancreatitis and kidney disease. Treatment with prednisolone for 2 months resulted in lesion improvement. Although the diagnosis of IgG4-related lymphadenopathy is often challenging in patients with lymphadenopathy alone, findings in the present patient emphasise the importance of long-term follow-up, as it may allow early detection of involvement of other organs by IgG4-related disease.