Bork Konrad, Hardt Jochen
Department of Dermatology, University Hospital Mainz, Mainz, Germany.
Am J Med. 2009 Aug;122(8):780-3. doi: 10.1016/j.amjmed.2009.02.024.
C1 inhibitor concentrate is regarded as effective and safe in treating acute attacks of hereditary angioedema caused by C1 inhibitor deficiency. This study investigated the course of disease in 3 women treated frequently with C1 inhibitor concentrate.
Three women are described who received C1 inhibitor concentrate for the treatment of acute attacks of hereditary angioedema and experienced an increase in the frequency of attacks during that treatment period. In a control group of 24 patients aged more than 60 years with hereditary angioedema, the natural course of disease was determined.
The 3 women (ages 50, 69, and 72 years) had received C1 inhibitor concentrate for 27, 18, and 22 years, respectively, for acute abdominal and skin attacks. Before this treatment, all attacks were severe. The treatment was always effective: The attacks were mild, and the duration of the attacks was shortened. During the treatment period, the number of attacks increased slowly but continuously, starting at the onset of treatment and paralleling the course of treatment. At the end of the observation period, the number of attacks increased by 4-fold, 12-fold, and 5-fold in the 3 women, respectively. No factors known to increase the frequency of attacks in hereditary angioedema were found in these patients. The control group did not show a similar increase in attacks during a comparable period of time.
A possible explanation for the increase in the frequency of attacks may lie in the large number of injections of C1 inhibitor concentrate.
C1抑制剂浓缩物被认为在治疗由C1抑制剂缺乏引起的遗传性血管性水肿急性发作方面有效且安全。本研究调查了3名经常接受C1抑制剂浓缩物治疗的女性的病程。
描述了3名女性，她们接受C1抑制剂浓缩物治疗遗传性血管性水肿急性发作，且在治疗期间发作频率增加。在一个由24名年龄超过60岁的遗传性血管性水肿患者组成的对照组中，确定了疾病的自然病程。
这3名女性（年龄分别为50岁、69岁和72岁）分别接受C1抑制剂浓缩物治疗急性腹部和皮肤发作27年、18年和22年。在这种治疗之前，所有发作都很严重。治疗总是有效的：发作轻微，发作持续时间缩短。在治疗期间，发作次数从治疗开始时起缓慢但持续增加，与治疗过程平行。在观察期结束时，这3名女性的发作次数分别增加了4倍、12倍和5倍。在这些患者中未发现已知会增加遗传性血管性水肿发作频率的因素。对照组在相当长的一段时间内未出现类似的发作增加情况。
发作频率增加的一个可能解释可能在于大量注射C1抑制剂浓缩物。
