Abstract
Objective
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.
Methods
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.
Results
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.
Conclusion
A possible explanation for the increase in the frequency of attacks may lie in the
large number of injections of C1 inhibitor concentrate.
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References
- Treatment of hereditary angioedema with a vapor-heated C1 inhibitor concentrate.N Engl J Med. 1996; 334: 1630-1634
- Treatment with C1 inhibitor concentrate in abdominal pain attacks of patients with hereditary angioedema.Transfusion. 2005; 45: 1774-1784
- Self-administration of C1-inhibitor concentrate in patients with hereditary or acquired angioedema caused by C1-inhibitor deficiency.J Allergy Clin Immunol. 2006; 117: 904-908
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- Pasteurized C1 inhibitor concentrate in hereditary angioedema: pharmacology, safety, efficacy and future directions.Exp Rev Clin Immunol. 2008; 4: 13-20
Article info
Footnotes
Funding: None.
Conflict of Interest: None.
Authorship: All authors had access to the data and played a role in writing this manuscript.
Identification
Copyright
© 2009 Elsevier Inc. Published by Elsevier Inc. All rights reserved.
ScienceDirect
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- C1 Inhibitor Therapy and Hereditary AngioedemaThe American Journal of MedicineVol. 123Issue 4
- PreviewWe read with interest the recent report by Bork and Hardt1 and would like to pose our thoughts to the authors. Certainly, a finding of increased attack frequency in hereditary angioedema patients being treated with C1 inhibitor (C1 INH) therapy is interesting and merits further study. However, a 3-subject review that preselects those patients with severe disease requiring C1 INH therapy on demand versus a retrospective historical control group that has never required any prophylactic therapy may not be the most ideal comparison.
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