国际口腔医学杂志 ›› 2019, Vol. 46 ›› Issue (4): 426-430.doi: 10.7518/gjkq.2019051

• 病例报告 • 上一篇    下一篇

抗中性粒细胞胞浆抗体阴性肉芽肿性多血管炎1例报告并文献复习

陈虹,吴兰雁,郑广宁,林梅()   

  1. 口腔疾病研究国家重点实验室 国家口腔疾病临床医学研究中心 四川大学华西口腔医院口腔黏膜病科 成都 610041
  • 收稿日期:2018-06-28 修回日期:2019-04-10 出版日期:2019-07-10 发布日期:2019-07-12
  • 作者简介:陈虹,博士,Email:709643911@qq.com

Seronegative granulomatosis with polyangiitis: a case report and literature review

Chen Hong,Wu Lanyan,Zheng Guangning,Lin Mei()   

  1. State Key Laboratory of Oral Diseases & National Clinical Research Center for Oral Diseases & Dept. of Oral Medicine, West China Hospital of Stomatology, Sichuan University, Chengdu 610041, China
  • Received:2018-06-28 Revised:2019-04-10 Online:2019-07-10 Published:2019-07-12

摘要:

肉芽肿性多血管炎(GPA)是一种以血管炎及坏死性肉芽肿为主要特征的全身系统性疾病。该病的临床表现复杂、多变,诊断需结合临床症状体征、抗中性粒细胞胞浆抗体(ANCA)检测及组织病理学检查结果。口腔草莓状牙龈损害是少见的黏膜病损。本文记录了1例仅有口腔和鼻腔损害的50岁男性藏族患者的诊断过程,其非典型的临床表现及ANCA阴性结果为诊断带来了较大的困难。旨在提醒广大医疗工作者在遇到相似临床表现时不应忽视GPA的排查。对局部口腔病变的早期诊断、早期治疗有重要临床价值,有助于改善患者的预后,对延缓病情进展有重要意义。

关键词: 肉芽肿性多血管炎, 病例报道, 文献分析, 抗中性粒细胞胞浆抗体

Abstract:

Granulomatosis with polyangiitis (GPA) is a systemic vasculitis and necrotising glomerulonephritis. The clinical manifestations of this condition are complex and varied, and the diagnosis requires the combined results of clinical symptoms, anti-neutrophil cytoplasmic antibody (ANCA) detection and histopathological examination. Strawberry gingivitis of GPA is rarely the reason of initial clinical presentation that is similar to inflammatory and infective diseases. This paper reports the case of a 50-year-old Chinese Tibetan patient with diagnostically difficult, ANCA-negative GPA that initially presented with oral and nasal manifestations only. The aim of this study is to remind dentists the possibility of GPA when faced with similar clinical manifestations. Moreover, early diagnosis and treatment play an important role in the improvement and prognosis of patients with GPA.

Key words: granulomatosis with polyangiitis, case report, literature review, anti-neutrophil cytoplasmic antibody

中图分类号: 

  • R781.5+9

图 1

初诊及复诊时患者的口内表现 A、B:初诊(2014-9-10);C:复诊(2014-10-8);D:随访3年(图片由患者家属提供,手机拍摄)。"

表 1

实验室检查结果"

项目 结果 参考值 项目 结果 参考值
白细胞计数/(×109 L-1 10.34 3.5~9.5 抗双链DNA抗体 阴性
红细胞计数/ (×1012 L-1 4.12 4.3~5.8 乙肝表面抗原/COI 1 867.000 -
血红蛋白/(g·L-1 149 130~175 乙肝表面抗体/COI 阴性
血小板计数/(×109 L-1 362 100~300 乙肝e抗原/COI 0.074 0~1
抗核抗体 阳性 阴性 乙肝e抗体/COI 0.004 ﹥1.00
CD3细胞亚群/ % 60.80 66.9~83.1 乙肝核心抗体/COI 0.005 ﹥1.00
CD4细胞亚群/ % 28.30 33.19~47.85 丙肝抗体/COI 阴性(0.033) ﹥1.00
CD8细胞亚群/ % 30.7 20.4~34.7 梅毒抗体 阴性(0.000) 0~1
CD4/CD8比值 0.92 0.97~2.31 梅毒特异性抗体/(s/co) 阴性(0.765)
CD4绝对计数/μL-1 448 471~1 220 人类免疫缺陷病毒抗体/(s/co) 阴性(0.069)
CD3绝对计数/μL-1 964 941~2 226 抗中性粒细胞浆抗体 阴性
CD8绝对计数 /μL-1 486 303~1 003 蛋白酶3 ﹤1.0 阴性
免疫球蛋白G 16.3 7~16.6 乳铁蛋白 ﹤1.0 ﹤1.0
补体C4 0.39 0.1~0.4 髓过氧化物酶 ﹤1.0 ﹤1.0
补体B因子 505 190~500 弹性蛋白酶 ﹤1.0 ﹤1.0
肾小球滤过率 阴性 组织蛋白酶 ﹤1.0 ﹤1.0
C-反应蛋白 阴性 杀菌性膜通透性蛋白 ﹤1.0 ﹤1.0

图 2

病例的全景片、CBCT及鼻部CTA、B、C:CBCT;D:全景片;E、F:鼻部CT。"

图 3

牙龈活组织检查 苏木精-伊红染色A、C:箭头显示大量炎细胞浸润;B:箭头显示血管炎;D:箭头显示多核巨细胞。"

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