Marco Cicardi, MD; Angelo Agostoni, MD; Alvin E. Davis, MD
Cicardi M., Agostoni A., Davis A.; Acquired C1 Esterase Inhibitor Deficiency. Ann Intern Med. 2000;133:837-838. doi: 10.7326/0003-4819-133-10-200011210-00022
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Published: Ann Intern Med. 2000;133(10):837-838.
TO THE EDITOR:
Markovic and colleagues' review (1) contains many unsupported statements that may promote inappropriate therapy. The authors' statements that corticosteroids, epinephrine, heparin, and antihistamines are effective therapy for acquired angioedema are misleading. No convincing published evidence suggests that any of these agents is effective therapy for either form of the disease. If the retrospective analysis of the patients treated at the Mayo Clinic indicates efficacy of these agents, these data should have been presented. In addition, both replacement therapy with C1 inhibitor concentrate and prophylaxis with androgen derivatives are frequently ineffective or marginally effective in acquired C1 inhibitor deficiency. This effect is probably secondary to anti–C1 inhibitor autoantibodies that interfere with C1 inhibitor function. The authors' description of the pathogenesis of angioedema ignores published evidence that the contact system mediates angioedema. As the authors summarize, the roles of C2 kinin and bradykinin have been debated for many years (2). Recent data confirmed findings from previous studies that indicated a major role for bradykinin. Contrary to the authors' comments, angiotensin-converting enzyme inhibitors clearly induce angioedema in patients with C1 inhibitor deficiency. This is consistent with bradykinin inactivation by angiotensin-converting enzyme. Markovic and colleagues also state that “all available published articles were reviewed and included in the manuscript.” The reference list, however, included only a small portion of the published literature on the subject and excludes at least two previous patient series. Finally, specific data were reported for only 2 of 22 patients reviewed. Information on the remainder of the patients is presented only in the form of conclusions, without reference to the data. The reader, therefore, cannot make an independent judgment of the information discussed.
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Print ISSN: 0003-4819 | Online ISSN: 1539-3704
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