title: Hereditary Angioedema Attacks: Local Swelling at Multiple Sites.
abstract: Hereditary angioedema (HAE) patients experience recurrent local swelling in various parts of the body including painful swelling of the intestine and life-threatening laryngeal oedema. Most HAE literature is about attacks located in one anatomical site, though it is mentioned that HAE attacks may also involve multiple anatomical sites simultaneously. A detailed description of such multi-location attacks is currently lacking. This study investigated the occurrence, severity and clinical course of HAE attacks with multiple anatomical locations. HAE patients included in a clinical database of recombinant human C1-inhibitor (rhC1INH) studies were evaluated. Visual analog scale scores filled out by the patients for various symptoms at various locations and investigator symptoms scores during the attack were analysed. Data of 219 eligible attacks in 119 patients was analysed. Thirty-three patients (28%) had symptoms at multiple locations in anatomically unrelated regions at the same time during their first attack. Up to five simultaneously affected locations were reported. The observation that severe HAE attacks often affect multiple sites in the body suggests that HAE symptoms result from a systemic rather than from a local process as is currently believed.
authors.affiliation: Laboratory for Translational Immunology, University Medical Center Utrecht, Lundlaan 6, P.O. Box 85090, 3508, GA, Utrecht, The Netherlands. zonnehofman@gmail.com.
authors.forename: Zonne L M
authors.initials: ZL
authors.lastname: Hofman
authors.affiliation: Pharming Technologies NV, Leiden, The Netherlands.
authors.forename: Anurag
authors.initials: A
authors.lastname: Relan
authors.affiliation: Laboratory for Translational Immunology, University Medical Center Utrecht, Lundlaan 6, P.O. Box 85090, 3508, GA, Utrecht, The Netherlands.
authors.forename: C Erik
authors.initials: CE
authors.lastname: Hack
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D054179:Angioedemas, Hereditary / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D015415:Biomarkers; D002097:C-Reactive Protein; D050718:Complement C1 Inhibitor Protein / Q000627:therapeutic use; D018450:Disease Progression; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011292:Premedication; D016032:Randomized Controlled Trials as Topic; D011994:Recombinant Proteins / Q000627:therapeutic use; D012720:Severity of Illness Index; D055815:Young Adult
chemical_list: D015415:Biomarkers; D050718:Complement C1 Inhibitor Protein; D011994:Recombinant Proteins; D002097:C-Reactive Protein
keywords: C1-inhibitor deficiency; Hereditary angioedema