Andy Kusuma, MD, Anurag Relan, MD, André C

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Clinical Impact of Peripheral Attacks in Hereditary Angioedema Patients  Andy Kusuma, MD, Anurag Relan, MD, André C. Knulst, MD, PhD, Dumitru Moldovan, MD, PhD, Bruce Zuraw, MD, Marco Cicardi, MD, Robyn J. Levy, MD, Jan H. Nuijens, MD, PhD, C. Erik Hack, MD, PhD  The American Journal of Medicine  Volume 125, Issue 9, Pages 937.e17-937.e24 (September 2012) DOI: 10.1016/j.amjmed.2011.12.016 Copyright © 2012 Elsevier Inc. Terms and Conditions

Figure 1 Hereditary angioedema patients with a peripheral attack frequently report pain and dysfunction in addition to swelling of the affected site. Patients were asked to score the severity of symptoms of the affected peripheral site using a 100-mm visual analog scale (VAS). Score (in mm) is given on the y-axis. Severe (≥ 50 mm), moderate (20-50 mm), and minimal symptoms (<20 mm) are indicated by shading. Squares=patients with one peripheral location of the attack; diamonds=patients with multiple peripheral locations; triangles=patients with one peripheral and at least one other location; circles=patients with multiple peripheral and at least on other location. The American Journal of Medicine 2012 125, 937.e17-937.e24DOI: (10.1016/j.amjmed.2011.12.016) Copyright © 2012 Elsevier Inc. Terms and Conditions

Figure 2 The course of the average overall severity and symptom visual analog scale (VAS) scores for the peripheral location in 36 symptomatic hereditary angioedema patients with an attack involving at least one peripheral location, who participated in the randomized controlled trials (RCT). VAS scores decreased more rapidly in the 22 patients who received recombinant human C1-inhibitor (rhC1INH; solid lines) than in the 14 patients who received saline. The different symbols indicate the different symptoms as explained in the inset. Bars indicate the standard error of the mean. The American Journal of Medicine 2012 125, 937.e17-937.e24DOI: (10.1016/j.amjmed.2011.12.016) Copyright © 2012 Elsevier Inc. Terms and Conditions

Figure 3 Recombinant human C1-inhibitor (rhC1INH) shortens the time to onset of relief of symptoms of a peripheral attack in hereditary angioedema patients. Shown are Kaplan-Meier plots of time (hours) to onset of relief of symptoms by overall severity and symptom-specific visual analog scale (VAS) in hereditary angioedema patients with an acute peripheral attack who received rhC1INH (solid lines) or saline (dotted lines). Note that only patients with a VAS ≥50 mm for the indicated symptom at baseline were included in this analysis. The number of patients included in each curve is indicated in the inset. The American Journal of Medicine 2012 125, 937.e17-937.e24DOI: (10.1016/j.amjmed.2011.12.016) Copyright © 2012 Elsevier Inc. Terms and Conditions

Figure 4 Recombinant human C1-inhibitor (rhC1INH) shortens the time to minimal symptoms of a peripheral attack in hereditary angioedema patients. Shown are Kaplan-Meier plots of time (hours) to minimal symptoms by overall severity and symptom-specific visual analog scale (VAS) in hereditary angioedema patients with an acute peripheral attack who received rhC1INH (solid lines) or saline (dotted lines). Note that only patients with a VAS ≥50 mm for the indicated symptom at baseline were included in this analysis. The number of patients included in each curve is indicated in the inset. The American Journal of Medicine 2012 125, 937.e17-937.e24DOI: (10.1016/j.amjmed.2011.12.016) Copyright © 2012 Elsevier Inc. Terms and Conditions