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肉芽肿性多血管炎(Wegener肉芽肿)的呼吸道受累

Author
Talmadge E King, Jr, MD
Section Editors
Kevin R Flaherty, MD, MS
Richard J Glassock, MD, MACP
Deputy Editor
Helen Hollingsworth, MD
Translators
孙雪峰, 主治医师

引言

“经典型”肉芽肿性多血管炎(Wegener肉芽肿)是系统性血管炎(多血管炎)的一种形式，伴有上呼吸道和下呼吸道的坏死性肉芽肿性炎症、系统性坏死性血管炎和坏死性肾小球肾炎[1,2]。“局限型”的临床表现局限于上呼吸道或肺，发生于约1/4的病例中。“局限型”这一名称主要是用于在治疗分层上与“重度”疾病相区分，并且不包单纯的抗中性粒细胞胞浆抗体(anti-neutrophil cytoplasmic antibodies, ANCA)相关性弥漫性肺泡出血(diffuse alveolar hemorrhage, DAH)[3]。许多“局限型”患者随后发生全身性受累[4]。

美国风湿病学会(American College of Rheumatology, ACR)、美国肾脏病学会(American Society of Nephrology, ASN)和欧洲抗风湿病联盟(European League Against Rheumatism, EULAR)的董事会已推荐将Wegener肉芽肿更名为肉芽肿性多血管炎(granulomatosis with polyangiitis, GPA，Wegener肉芽肿)，缩写为GPA。 (参见“肉芽肿性多血管炎(Wegener’s)和显微镜下多血管炎的临床表现和诊断”，关于‘新术语’一节)

GPA可能累及的其他器官系统包括[1]：

  • 关节(肌痛、关节痛、关节炎)
  • 眼睛(结膜炎、表层巩膜炎、葡萄膜炎)
  • 皮肤(水泡性、紫癜性和出血性病变、皮下结节)
  • 神经系统(多发性单神经炎、颅神经异常、眼外肌麻痹、耳鸣、听力损失)
  • 心脏(心包炎、心肌炎、传导系统异常)
  • 更少见情况下，胃肠道、下泌尿生殖道(包括前列腺或输尿管)、腮腺、甲状腺、肝脏或乳房受累

GPA中呼吸道受累相关的主要特征将总结在此。GPA及相关疾病显微镜下多血管炎的发病机制、临床表现、诊断、治疗及结局的相关问题将单独讨论。 (参见“肉芽肿性多血管炎及相关血管炎的发病机制”和“肉芽肿性多血管炎(Wegener’s)和显微镜下多血管炎的临床表现和诊断”和“肉芽肿性多血管炎和显微镜下多血管炎的免疫抑制初始治疗”和“肉芽肿性多血管炎和显微镜下多血管炎的免疫抑制维持治疗”和“肉芽肿性血管炎和显微镜下多血管炎的预后及出现终末期肾病患者的处理”)

                               

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Literature review current through: 2017-07 . | This topic last updated: 2016-08-30.
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