Efficacy of Treatment of Non-hereditary Angioedema.
Non-hereditary angioedema (AE) with normal C1 esterase inhibitor (C1INH) can be presumably bradykinin- or mast cell-mediated, or of unknown cause. In this systematic review, we searched PubMed, EMBASE, and Scopus to provide an overview of the efficacy of different treatment options for the abovementioned subtypes of refractory non-hereditary AE with or without wheals and with normal C1INH. After study selection and risk of bias assessment, 61 articles were included for data extraction and analysis. Therapies were described for angiotensin-converting enzyme inhibitor-induced AE (ACEi-AE), for idiopathic AE, and for AE with wheals. Described treatments consisted of ecallantide, icatibant, C1INH, fresh frozen plasma (FFP), tranexamic acid (TA), and omalizumab. Additionally, individual studies for anti-vitamin K, progestin, and methotrexate were found. Safety information was available in 26 articles. Most therapies were used off-label and in few patients. There is a need for additional studies with a high level of evidence. In conclusion, in acute attacks of ACEi-AE and idiopathic AE, treatment with icatibant, C1INH, TA, and FFP often leads to symptom relief within 2 h, with limited side effects. For prophylactic treatment of idiopathic AE and AE with wheals, omalizumab, TA, and C1INH were effective and safe in the majority of patients.
Division of Internal Medicine and Dermatology, University Medical Center Utrecht, Utrecht, The Netherlands. melzen3@umcutrecht.nl.|Department of Dermatology and Allergology, University Medical Center Utrecht (G02.124), PO Box 85.500, 3508 GA, Utrecht, The Netherlands. melzen3@umcutrecht.nl.
Mignon
http://orcid.org/0000-0002-8796-2231
M
van den Elzen
Division of Internal Medicine and Dermatology, University Medical Center Utrecht, Utrecht, The Netherlands.
M F C L
MFCL
Go
Division of Internal Medicine and Dermatology, University Medical Center Utrecht, Utrecht, The Netherlands.
A C
AC
Knulst
Division of Internal Medicine and Dermatology, University Medical Center Utrecht, Utrecht, The Netherlands.
M A
MA
Blankestijn
Division of Internal Medicine and Dermatology, University Medical Center Utrecht, Utrecht, The Netherlands.
H
H
van Os-Medendorp
Laboratory of Translational Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
H G
HG
Otten
D000799:Angioedema / Q000628:therapy*; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use*; D001920:Bradykinin / Q000031:analogs & derivatives* / Q000627:therapeutic use; D006801:Humans; D000069444:Omalizumab / Q000627:therapeutic use*; D011372:Progestins / Q000627:therapeutic use; D014148:Tranexamic Acid / Q000627:therapeutic use*; D016896:Treatment Outcome
D000806:Angiotensin-Converting Enzyme Inhibitors; D011372:Progestins; D000069444:Omalizumab; D014148:Tranexamic Acid; C065679:icatibant; D001920:Bradykinin
Angioedema; Angiotensin-converting enzyme inhibitor; Idiopathic; Treatment; Wheals
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