Immunoglobulin G4-Related Appendicitis.

Immunoglobulin G4-Related Appendicitis.
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DOI:
10.1097/rhu.0000000000001151
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发表时间:
2020-12
期刊:
JCR: Journal of Clinical Rheumatology
影响因子:
--
通讯作者:
Ying Gao;Dongbao Zhao;Lianjie Liu
Ying Gao;Dongbao Zhao;Lianjie Liu
中科院分区:
其他
文献类型:
--
作者:
Ying Gao;Dongbao Zhao;Lianjie Liu

文献摘要

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患者男,25岁,因腹胀腹痛、高热、体重迅速下降1个月就诊。呕吐后症状消退。体格检查时,我们在右髂部触诊到一个肿块。检查显示红细胞沉降率为60 mm/H(参考范围,0-15 mm/H),血清淀粉酶水平升高为368 U/L(参考范围,20-90 U/L)。腹部计算机断层扫描(CT)显示阑尾周围脓肿(图1A)和小肠梗阻(图1B)。不幸的是,病人对包括亚胺培南在内的抗生素治疗没有反应,我们坚持在进行紧急阑尾切除术前获得病理学证据,因为阑尾周围的肿块有脂肪模糊。我们没有通过CT引导穿刺排出任何脓液;因此,我们不得不采集一些肿块组织进行活检。然而,苏木精-伊红染色显示浆细胞浸润和丝状纤维化(图2A)。免疫球蛋白G4(IgG 4)染色显示IgG 4/IgG比率超过40%(图2B)。另一项检测显示血清IgG 4水平高达8.6 g/L。根据血清IgG 4水平升高、阑尾肿块和典型病理学证据,我们诊断该患者为IgG 4相关性阑尾炎。患者接受甲泼尼龙20 mg/d治疗,反应良好。3天后患者在直立位进行的随访腹部X线片证实了疗效。此外,血清IgG 4和淀粉酶水平分别降至2.2 g/L和114 U/L。到
A 25-year-old young man presented with 1 month's history of abdominal distension and pain, high fever, and rapid weight loss. Symptoms resolved after vomiting. On physical examination, we palpated a mass in the right iliac region. The tests showed an erythrocyte sedimentation rate of 60 mm/H (reference range, 0–15 mm/H) and elevated serum amylase level of 368 U/L (reference range, 20–90 U/L). Abdominal computed tomography (CT) revealed a periappendicular abscess (Fig. 1A) and small intestinal obstructions (Fig. 1B). Unfortunately, the patient did not respond to the administration of antibiotics, including imipenem.We insisted on obtaining pathological evidence before performing an emergency appendectomy because there was fat haziness around the appendicular mass. We did not drain out any pus by CT-guided puncture; therefore, we had to sample some mass tissue for biopsy. However, hematoxylin-eosin staining showed plasmacytic infiltration and storiform fibrosis (Fig. 2A). Immunoglobulin G4 (IgG4) staining showed that the IgG4/IgG ratio was over 40% (Fig. 2B). An additional test revealed the serum IgG4 level to be as high as 8.6 g/L. Based on the findings of an elevated serum IgG4 level, mass in the appendix, and typical pathologic evidence, we diagnosed the patient with IgG4-related appendicitis. The patient receivedmethylprednisolone 20 mg/d and responded well. A follow-up abdominal x-ray film performed 3 days later with the patient in an erect position verified the efficacy. Moreover, the serum IgG4 and amylase levels decreased to 2.2 g/L and 114 U/L, respectively. To