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Acquired C1 Esterase Inhibitor Deficiency
- Svetomir N. Markovic, MD, PhD;
- David J. Inwards, MD; and
- Robert P. Phyliky, MD
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IN RESPONSE:
Caballero and colleagues take issue with the two cases illustrating acquired C1 esterase deficiency and suggest the need for greater detail in the description of these cases. They also appear to have anticipated a full description of all 22 cases identified in our database. We appreciate the comments, but we felt that a detailed presentation of all 22 cases would have been inappropriate for the format of our manuscript. The two presented cases were intended to illustrate the typical course of acquired C1 esterase inhibitor deficiency. Caballero and colleagues favor the use of intravenous C1 inhibitor concentrate “where available.” Given the unavailability of this agent in general practice (at least in the United States) and the efficacy of other therapies, we believe that the described management of acquired C1 esterase inhibitor deficiency, successfully used in our institution, is a reasonable approach. Concerning our diagnostic work-up flow chart, we favor screening with commonly available tests, leaving more sophisticated and more expensive testing for confirmation of diagnosis. Therefore, we usually elect not to measure the C1 esterase inhibitor function when screening for this disorder. Cicardi and coworkers comment on the lack of convincing evidence that the agents used in our practice to control symptoms of acquired C1 esterase inhibitor deficiency are effective. Part of the goal of our review was to share our clinical experience with the use of these agents in the successful management of acquired C1 esterase inhibitor deficiency. In our experience, androgen derivatives are effective in the management of acquired C1 esterase deficiency.
The role of bradykinins (as mentioned by Kaplan and Cicardi and colleagues) as mediators of angioedema in this disorder has been debated for years. Some data support the role of both bradykinin and C2 kinin. Although we did not intend to elaborate on this debate, we accept Dr. Kaplan's suggestions and should have commented on the role of bradykinins as possible mediators of angioedema in acquired C1 esterase inhibitor deficiency.
Csepregi and Nemesánszky comment on the recently published description of two patients who developed acquired C1 esterase inhibitor deficiency in the setting of hepatitis C. Unfortunately, this reference was not available at the time of submission of our manuscript.
Svetomir N. Markovic, MD, PhD
David J. Inwards, MD
Robert P. Phyliky, MD
Mayo Clinic; Rochester, MN 55905
- Copyright ©2004 by the American College of Physicians
