U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Angioedema, Hereditary, Type II(HAE2)

MedGen UID:
400156
•Concept ID:
C1862892
•
Disease or Syndrome
Synonym: Hereditary Angioedema Type II
 
OMIM®: 106100; 606860

Professional guidelines

PubMed

Isolated angioedema: A review of classification and update on management.
Kesh S, Bernstein JA
Ann Allergy Asthma Immunol 2022 Dec;129(6):692-702. Epub 2022 Aug 19 doi: 10.1016/j.anai.2022.08.003. PMID: 35988876
Hereditary angioedema: an update on causes, manifestations and treatment.
Longhurst HJ, Bork K
Br J Hosp Med (Lond) 2019 Jul 2;80(7):391-398. doi: 10.12968/hmed.2019.80.7.391. PMID: 31283393
Pharmacological Management of Hereditary Angioedema with C1-Inhibitor Deficiency in Pediatric Patients.
Farkas H
Paediatr Drugs 2018 Apr;20(2):135-151. doi: 10.1007/s40272-017-0273-x. PMID: 29214395

Recent clinical studies

Therapy

Efficacy and safety of garadacimab, a factor XIIa inhibitor for hereditary angioedema prevention (VANGUARD): a global, multicentre, randomised, double-blind, placebo-controlled, phase 3 trial.
Craig TJ, Reshef A, Li HH, Jacobs JS, Bernstein JA, Farkas H, Yang WH, Stroes ESG, Ohsawa I, Tachdjian R, Manning ME, Lumry WR, Saguer IM, Aygören-Pürsün E, Ritchie B, Sussman GL, Anderson J, Kawahata K, Suzuki Y, Staubach P, Treudler R, Feuersenger H, Glassman F, Jacobs I, Magerl M
Lancet 2023 Apr 1;401(10382):1079-1090. Epub 2023 Feb 28 doi: 10.1016/S0140-6736(23)00350-1. PMID: 36868261
Interventions for the long-term prevention of hereditary angioedema attacks.
Beard N, Frese M, Smertina E, Mere P, Katelaris C, Mills K
Cochrane Database Syst Rev 2022 Nov 3;11(11):CD013403. doi: 10.1002/14651858.CD013403.pub2. PMID: 36326435Free PMC Article
Isolated angioedema: A review of classification and update on management.
Kesh S, Bernstein JA
Ann Allergy Asthma Immunol 2022 Dec;129(6):692-702. Epub 2022 Aug 19 doi: 10.1016/j.anai.2022.08.003. PMID: 35988876
Effect of Lanadelumab Compared With Placebo on Prevention of Hereditary Angioedema Attacks: A Randomized Clinical Trial.
Banerji A, Riedl MA, Bernstein JA, Cicardi M, Longhurst HJ, Zuraw BL, Busse PJ, Anderson J, Magerl M, Martinez-Saguer I, Davis-Lorton M, Zanichelli A, Li HH, Craig T, Jacobs J, Johnston DT, Shapiro R, Yang WH, Lumry WR, Manning ME, Schwartz LB, Shennak M, Soteres D, Zaragoza-Urdaz RH, Gierer S, Smith AM, Tachdjian R, Wedner HJ, Hebert J, Rehman SM, Staubach P, Schranz J, Baptista J, Nothaft W, Maurer M; HELP Investigators
JAMA 2018 Nov 27;320(20):2108-2121. doi: 10.1001/jama.2018.16773. PMID: 30480729Free PMC Article
Angioedema.
Hahn J, Hoffmann TK, Bock B, Nordmann-Kleiner M, Trainotti S, Greve J
Dtsch Arztebl Int 2017 Jul 24;114(29-30):489-496. doi: 10.3238/arztebl.2017.0489. PMID: 28818177Free PMC Article

Prognosis

Hereditary Angioedema Type 1 and 2 in Finland: Incidence, Prevalence, and Preceding Diagnoses.
Sandberg A, Lassenius M, Vihervaara V, Toppila I, Huilaja L
Acta Derm Venereol 2024 May 6;104:adv24176. doi: 10.2340/actadv.v104.24176. PMID: 38708992Free PMC Article
The analysis of the effect of the COVID-19 pandemic on patients with hereditary angioedema type I and type II.
Szilágyi D, Horváth HR, Andrási N, Kempler MS, Balla Z, Farkas H
Sci Rep 2023 Nov 22;13(1):20446. doi: 10.1038/s41598-023-47307-1. PMID: 37993569Free PMC Article
COVID-19 and hereditary angioedema: Incidence, outcomes, and mechanistic implications.
Veronez CL, Christiansen SC, Smith TD, Riedl MA, Zuraw BL
Allergy Asthma Proc 2021 Nov 1;42(6):506-514. doi: 10.2500/aap.2021.42.210083. PMID: 34871158Free PMC Article
"Nuts and Bolts" of Laboratory Evaluation of Angioedema.
Farkas H, Veszeli N, Kajdácsi E, Cervenak L, Varga L
Clin Rev Allergy Immunol 2016 Oct;51(2):140-51. doi: 10.1007/s12016-016-8539-6. PMID: 27142368
Overview of hereditary angioedema caused by C1-inhibitor deficiency: assessment and clinical management.
Bork K, Davis-Lorton M
Eur Ann Allergy Clin Immunol 2013 Feb;45(1):7-16. PMID: 23678554

Clinical prediction guides

HAE Pathophysiology and Underlying Mechanisms.
Zuraw BL, Christiansen SC
Clin Rev Allergy Immunol 2016 Oct;51(2):216-29. doi: 10.1007/s12016-016-8561-8. PMID: 27459852
Type I and Type II Hereditary Angioedema: Clinical and Laboratory Findings in Iranian Patients.
Kargarsharif F, Mehranmehr N, Zahedi Fard S, Fazlollahi MR, Ayazi M, Mohammadzadeh I, Nabavi M, Bemanian MH, Fayezi A, Movahedi M, Heidarzadeh M, Kalantari N, Arefimehr S, Saghafi S, Pourpak Z
Arch Iran Med 2015 Jul;18(7):425-9. PMID: 26161706
Clinical features of pediatric hereditary angioedema.
Nanda MK, Elenburg S, Bernstein JA, Assa'ad AH
J Allergy Clin Immunol Pract 2015 May-Jun;3(3):392-5. Epub 2015 Jan 15 doi: 10.1016/j.jaip.2014.11.012. PMID: 25609346Free PMC Article
Overview of hereditary angioedema caused by C1-inhibitor deficiency: assessment and clinical management.
Bork K, Davis-Lorton M
Eur Ann Allergy Clin Immunol 2013 Feb;45(1):7-16. PMID: 23678554
Human C1-esterase inhibitor concentrate (Berinert).
Keating GM
BioDrugs 2009;23(6):399-406. doi: 10.2165/11201100-000000000-00000. PMID: 19894781

Recent systematic reviews

Interventions for the long-term prevention of hereditary angioedema attacks.
Beard N, Frese M, Smertina E, Mere P, Katelaris C, Mills K
Cochrane Database Syst Rev 2022 Nov 3;11(11):CD013403. doi: 10.1002/14651858.CD013403.pub2. PMID: 36326435Free PMC Article
Psychology and hereditary angioedema: A systematic review.
Savarese L, Mormile I, Bova M, Petraroli A, Maiello A, Spadaro G, Freda MF
Allergy Asthma Proc 2021 Jan 1;42(1):e1-e7. doi: 10.2500/aap.2021.42.200073. PMID: 33404395Free PMC Article
Treatment with C1-esterase inhibitor concentrate in type I or II hereditary angioedema: a systematic literature review.
Bork K, Steffensen I, Machnig T
Allergy Asthma Proc 2013 Jul-Aug;34(4):312-327. Epub 2013 May 22 doi: 10.2500/aap.2013.34.3677. PMID: 23710659

Supplemental Content

Table of contents

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Related information

    • MeSH
      Related Medical Subject Headings
    • OMIM
      Related records in OMIM
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed
    • PubMed (OMIM)
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...