Kardum Željka, Prus Višnja, Milas Ahić Jasminka, Kardum Darjan
Department of Rheumatology, Clinical Immunology and Allergology, University Hospital Osijek, J. Huttlera 4, 31000, Osijek, Croatia.
School of Medicine, Josip Juraj Strossmayer University of Osijek J, Huttlera 4, 31000, Osijek, Croatia.
J Med Case Rep. 2021 Jan 24;15(1):20. doi: 10.1186/s13256-020-02622-3.
Hereditary angioedema (HAE) is a rare disease characterized with recurrent swelling of subcutaneous or mucosal tissue that resolves in approximately 3 days. It can be presented with peripheral edema, abdominal and life-threatening laryngeal angioedema. A variety of triggers are known to cause episodes of angioedema including estrogen exposure. There are different reports regarding the effect of pregnancy on HAE attacks, and in some patients, the pregnancy is a recognized triggering factor.
We present a female Caucasian patient with pre-existing HAE and disease exacerbations during pregnancy, requiring prophylactic use of plasma-derived C1 inhibitor concentrate. She was treated with Cinryze® replacement therapy throughout the pregnancy 1000 IU i.v. 48 times. She gave birth to a healthy male infant, via C-section. After the delivery, the patient was symptom-free for 6 months and required no treatment for HAE.
In the case presented, the angioedema attacks worsened as the pregnancy progressed. The treatment with Cinryze® replacement therapy was effective and safe during pregnancy, with no adverse effects on the infant.
遗传性血管性水肿（HAE）是一种罕见疾病，其特征为皮下或黏膜组织反复肿胀，约3天可消退。可表现为外周水肿、腹部血管性水肿及危及生命的喉部血管性水肿。已知多种触发因素可导致血管性水肿发作，包括接触雌激素。关于妊娠对HAE发作的影响有不同报道，在一些患者中，妊娠是公认的触发因素。
我们报告一名患有HAE的白种女性患者，孕期病情加重，需要预防性使用血浆源性C1抑制剂浓缩物。整个孕期她接受了Cinryze®替代疗法，静脉注射1000 IU，共48次。她通过剖宫产分娩出一名健康男婴。产后，患者6个月无症状，无需治疗HAE。
在本病例中，血管性水肿发作随妊娠进展而加重。孕期使用Cinryze®替代疗法有效且安全，对婴儿无不良影响。
