Nadeem Ifrah, Khan Dawlat, Huang Jiannan, Aslam Sadia, Nadeem Anum, Khan Wahab J
Internal Medicine, University of South Dakota Sanford School of Medicine, Sioux Falls, USA.
Internal Medicine, Avera McKennan Hospital and University Health Center, Sioux Falls, USA.
Cureus. 2023 May 17;15(5):e39141. doi: 10.7759/cureus.39141. eCollection 2023 May.
Hereditary angioedema (HAE) is an autosomal dominant condition marked by a lack of functioning C1 esterase inhibitor (C1-INH). In contrast, acquired angioedema (AAE) due to a deficiency of C1 esterase inhibitor (AAE-C1-INH) may be the manifestation of an underlying lymphoproliferative, neoplastic, or autoimmune condition. Both are potentially fatal. The C1q protein is normal in HAE but low in AAE. A third mechanism has been reported to cause angioedema, especially in systemic lupus erythematosus (SLE) patients. AAE, which happens in association with SLE, may respond well to steroids. Here we present a case of AAE in a young female with SLE that led to upper airway compromise, requiring endotracheal intubation. Early detection and treatment of such cases can lead to an outstanding prognosis by preventing airway compromise and anoxic brain injury. Even though it is a condition of either very young or middle-aged patients, practitioners must be aware of this uncommon disease linked with SLE in adolescents and young adults.
遗传性血管性水肿（HAE）是一种常染色体显性疾病，其特征是缺乏具有功能的C1酯酶抑制剂（C1-INH）。相比之下，由于C1酯酶抑制剂缺乏导致的获得性血管性水肿（AAE-C1-INH）可能是潜在的淋巴增生性、肿瘤性或自身免疫性疾病的表现。两者都有潜在的致命性。C1q蛋白在HAE中正常，但在AAE中较低。据报道，还有第三种机制可导致血管性水肿，尤其是在系统性红斑狼疮（SLE）患者中。与SLE相关的AAE可能对类固醇治疗反应良好。在此，我们报告一例年轻女性SLE患者发生AAE，导致上呼吸道梗阻，需要进行气管插管。早期发现并治疗此类病例，通过预防气道梗阻和缺氧性脑损伤，可带来良好的预后。尽管这是一种多见于非常年轻或中年患者的疾病，但从业者必须了解这种与青少年和年轻成人SLE相关的罕见疾病。
