Preferred Therapy for Patients with Hereditary Angioedema during Pregnancy
Abstract
:1. Introduction
2. HAE during Pregnancy and Lactation
3. Long-Term Prophylaxis
4. Short-Term Prophylaxis
5. Recommendations
6. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
- Longhurst, H.J.; Bork, K. Hereditary Angioedema: An Update on Causes, Manifestations and Treatment. Br. J. Hosp. Med. 2019, 80, 391–398. [Google Scholar] [CrossRef]
- Cicardi, M.; Aberer, W.; Banerji, A.; Bas, M.; Bernstein, J.A.; Bork, K.; Caballero, T.; Farkas, H.; Grumach, A.; Kaplan, A.P.; et al. Classification, Diagnosis, and Approach to Treatment for Angioedema: Consensus Report from the Hereditary Angioedema International Working Group. Allergy 2014, 69, 602–616. [Google Scholar] [CrossRef] [Green Version]
- Lumry, W.R. Hereditary Angioedema: The Economics of Treatment of an Orphan Disease. Front. Med. 2018, 5, 22. [Google Scholar] [CrossRef] [PubMed] [Green Version]
- Nussberger, J.; Cugno, M.; Amstutz, C.; Cicardi, M.; Pellacani, A.; Agostoni, A. Plasma Bradykinin in Angio-Oedema. Lancet 1998, 351, 1693–1697. [Google Scholar] [CrossRef] [PubMed]
- Maurer, M.; Magerl, M.; Betschel, S.; Aberer, W.; Ansotegui, I.J.; Aygören-Pürsün, E.; Banerji, A.; Bara, N.; Boccon-Gibod, I.; Bork, K.; et al. The International WAO/EAACI Guideline for the Management of Hereditary Angioedema—The 2021 Revision and Update. Allergy 2022, 77, 1961–1990. [Google Scholar] [CrossRef]
- Davis, A.E. The Pathophysiology of Hereditary Angioedema. Clin. Immunol. 2005, 114, 3–9. [Google Scholar] [CrossRef]
- Azmy, V.; Brooks, J.P.; Hsu, F.I. Clinical Presentation of Hereditary Angioedema. Allergy Asthma Proc. 2020, 41, S18–S21. [Google Scholar] [CrossRef]
- Minafra, F.G.; Gonçalves, T.R.; Alves, T.M.; Pinto, J.A. The Mortality from Hereditary Angioedema Worldwide: A Review of the Real-World Data Literature. Clinic. Rev. Allerg. Immunol. 2022, 62, 232–239. [Google Scholar] [CrossRef]
- Maurer, M.; Magerl, M. Hereditary Angioedema: An Update on Available Therapeutic Options. J. Dtsch. Dermatol. Ges. 2010, 8, 663–672. [Google Scholar] [CrossRef]
- Nicola, S.; Rolla, G.; Brussino, L. Breakthroughs in Hereditary Angioedema Management: A Systematic Review of Approved Drugs and Those under Research. Drugs Context 2019, 8, 1–11. [Google Scholar] [CrossRef]
- Valerieva, A.; Nedeva, D.; Yordanova, V.; Petkova, E.; Staevska, M. Therapeutic Management of Hereditary Angioedema: Past, Present, and Future. Balkan Med. J. 2021, 38, 89–103. [Google Scholar] [CrossRef]
- Farkas, H.; Zotter, Z.; Csuka, D.; Szabó, E.; Nébenfűhrer, Z.; Temesszentandrási, G.; Jakab, L.; Varga, L.; Harmat, G.; Karádi, I. Short-Term Prophylaxis in Hereditary Angioedema due to Deficiency of the C1-Inhibitor—A Long-Term Survey. Allergy 2012, 67, 1586–1593. [Google Scholar] [CrossRef] [PubMed]
- Zotter, Z.; Csuka, D.; Szabó, E.; Czaller, I.; Nébenführer, Z.; Temesszentandrási, G.; Fust, G.; Varga, L.; Farkas, H. The Influence of Trigger Factors on Hereditary Angioedema due to C1-Inhibitor Deficiency. Orphanet J. Rare Dis. 2014, 9, 44. [Google Scholar] [CrossRef] [PubMed] [Green Version]
- Bouillet, L.; Longhurst, H.; Boccon-Gibod, I.; Bork, K.; Bucher, C.; Bygum, A.; Caballero, T.; Drouet, C.; Farkas, H.; Massot, C.; et al. Disease Expression in Women with Hereditary Angioedema. Am. J. Obstet. Gynecol. 2008, 199, 484.e1–484.e4. [Google Scholar] [CrossRef] [PubMed]
- Banerji, A.; Riedl, M. Managing the Female Patient with Hereditary Angioedema. Women’s Health 2016, 12, 351–361. [Google Scholar] [CrossRef] [Green Version]
- Gabriel, N.; Marcelino, F.; Ferriani, M.P.L.; Arruda, L.K.; Campos, R.A.; Gonçalves, R.F.; Chong-Neto, H.; Rosario Filho, N.; Valle, S.O.R.; Pesquero, J.B.; et al. Pregnancy in Patients with Hereditary Angioedema and Normal C1 Inhibitor. Front. Allergy 2022, 3. [Google Scholar] [CrossRef]
- Caballero, T.; Canabal, J.; Rivero-Paparoni, D.; Cabanas, R. Management of Hereditary Angioedema in Pregnant Women: A Review. Int. J. Womens Health 2014, 839. [Google Scholar] [CrossRef] [Green Version]
- Bork, K.; Meng, G.; Staubach, P.; Hardt, J. Hereditary Angioedema: New Findings Concerning Symptoms, Affected Organs, and Course. Am. J. Med. 2006, 119, 267–274. [Google Scholar] [CrossRef]
- Bork, K.; Wulff, K.; Hardt, J.; Witzke, G.; Staubach, P. Hereditary Angioedema Caused by Missense Mutations in the Factor XII Gene: Clinical Features, Trigger Factors, and Therapy. J. Allergy Clin. Immunol. 2009, 124, 129–134. [Google Scholar] [CrossRef]
- Marcos, C.; López Lera, A.; Varela, S.; Liñares, T.; Alvarez-Eire, M.G.; López-Trascasa, M. Clinical, Biochemical, and Genetic Characterization of Type III Hereditary Angioedema in 13 Northwest Spanish Families. Ann. Allergy Asthma Immunol. 2012, 109, 195–200. [Google Scholar] [CrossRef]
- Walker, J.P.; Campbell, D.K.; Ogston, D. Blood Levels of Proteinase Inhibitors in Pregnancy. Br. J. Obstet. Gynaecol. 1982, 89, 208–210. [Google Scholar] [CrossRef] [PubMed]
- Halbmayer, W.M.; Hopmeier, P.; Mannhalter, C.; Heuss, F.; Leodolter, S.; Rubi, K.; Fischer, M. C1-Esterase Inhibitor in Uncomplicated Pregnancy and Mild and Moderate Preeclampsia. Thromb. Haemost. 1991, 65, 134–138. [Google Scholar] [CrossRef] [PubMed]
- Cohen, A.; Laskin, C.A.; Tarlo, S.M. C1 Esterase Inhibitor in Pregnancy. J. Allergy Clin. Immunol. 1992, 90, 412–413. [Google Scholar] [CrossRef]
- Caballero, T.; Farkas, H.; Bouillet, L.; Bowen, T.; Gompel, A.; Fagerberg, C.; Bjökander, J.; Bork, K.; Bygum, A.; Cicardi, M.; et al. International Consensus and Practical Guidelines on the Gynecologic and Obstetric Management of Female Patients with Hereditary Angioedema Caused by C1 Inhibitor Deficiency. J. Allergy Clin. Immunol. 2012, 129, 308–320. [Google Scholar] [CrossRef] [PubMed]
- Zuraw, B.L.; Christiansen, S.C. HAE Pathophysiology and Underlying Mechanisms. Clin. Rev. Allergy Immunol. 2016, 51, 216–229. [Google Scholar] [CrossRef] [PubMed]
- Margaglione, M.; D’Apolito, M.; Santocroce, R.; Maffione, A.B. Hereditary Angioedema: Looking for Bradykinin Production and Triggers of Vascular Permeability. Clin. Exp. Allergy 2019, 49, 1395–1402. [Google Scholar] [CrossRef]
- Chair, I.I.; Binkley, K.E.; Betschel, S. Hereditary Angioedema in Pregnancy. Obstet. Gynecol. Surv. 2021, 76, 566–574. [Google Scholar] [CrossRef]
- Sundberg, K.; Bang, J.; Smidt-Jensen, S.; Brocks, V.; Lundsteen, C.; Parner, J.; Keiding, N.; Philip, J. Randomised Study of Risk of Fetal Loss Related to Early Amniocentesis versus Chorionic Villus Sampling. Lancet 1997, 350, 697–703. [Google Scholar] [CrossRef]
- Farkas, H.; Martinez-Saguer, I.; Bork, K.; Bowen, T.; Craig, T.; Frank, M.; Germenis, A.E.; Grumach, A.S.; Luczay, A.; Varga, L.; et al. International Consensus on the Diagnosis and Management of Pediatric Patients with Hereditary Angioedema with C1 Inhibitor Deficiency. Allergy 2016, 72, 300–313. [Google Scholar] [CrossRef] [Green Version]
- Nielsen, E.W.; Johansen, H.T.; Holt, J.; Mollnes, T.E. C1 Inhibitor and Diagnosis of Hereditary Angioedema in Newborns. Pediatr. Res. 1994, 35, 184–187. [Google Scholar] [CrossRef] [Green Version]
- Davis, C.A.; Vallota, E.H.; Forristal, J. Serum Complement Levels in Infancy: Age Related Changes. Pediatr. Res. 1979, 13, 1043–1046. [Google Scholar] [CrossRef] [PubMed] [Green Version]
- Agostoni, A.; Aygören-Pürsün, E.; Binkley, K.E.; Blanch, A.; Bork, K.; Bouillet, L.; Bucher, C.; Castaldo, A.J.; Cicardi, M.; Davis, A.E.; et al. Hereditary and Acquired Angioedema: Problems and Progress: Proceedings of the Third C1 Esterase Inhibitor Deficiency Workshop and Beyond. J. Allergy Clin. Immunol. 2004, 114, S51–S131. [Google Scholar] [CrossRef] [PubMed]
- Caballero, T.; Baeza, M.L.; Cabañas, R.; Campos, A.; Cimbollek, S.; Gómez-Traseira, C.; González-Quevedo, T.; Guilarte, M.; Jurado-Palomo, G.J.; Larco, J.I.; et al. Consensus Statement on the Diagnosis, Management, and Treatment of Angioedema Mediated by Bradykinin. Part I. Classification, Epidemiology, Pathophysiology, Genetics, Clinical Symptoms, and Diagnosis. J. Investig. Allergol. Clin. Immunol. 2011, 21, 333–347. [Google Scholar] [PubMed]
- Betschel, S.; Badiou, J.; Binkley, K.; Borici-Mazi, R.; Hébert, J.; Kanani, A.; Keith, P.; Lacuesta, G.; Waserman, S.; Yang, B.; et al. The International/Canadian Hereditary Angioedema Guideline. Allergy Asthma Clin. Immunol. 2019, 15. [Google Scholar] [CrossRef] [PubMed] [Green Version]
- González-Quevedo, T.; Larco, J.; Marcos, C.; Guilarte, M.; Baeza, M.; Cimbollek, S.; López-Serrano, M.; Piñero-Saavedra, M.; Rubio, M.; Caballero, T. Management of Pregnancy and Delivery in Patients with Hereditary Angioedema due to C1 Inhibitor Deficiency. J. Investig. Allergol. Clin. Immunol. 2016, 26, 161–167. [Google Scholar] [CrossRef] [PubMed] [Green Version]
- Satomura, A.; Fujita, T.; Nakayama, T. Comparison of the Frequency of Angioedema Attack, before and during Pregnancy, in a Patient with Type I Hereditary Angioedema. Intern. Med. 2018, 57, 751–755. [Google Scholar] [CrossRef] [Green Version]
- Chan, W.; Berlin, N.; Sussman, G.L. Management of Hereditary Angioedema with C1-Inhibitor Concentrate during Two Successive Pregnancies. Int. J. Gynaecol. Obstet. 2012, 120, 189–190. [Google Scholar] [CrossRef]
- Czaller, I.; Visy, B.; Csuka, D.; Füst, G.; Tóth, F.; Farkas, H. The Natural History of Hereditary Angioedema and the Impact of Treatment with Human C1-Inhibitor Concentrate during Pregnancy: A Long-Term Survey. Eur. J. Obstet. Gynecol. Reprod. Biol. 2010, 152, 44–49. [Google Scholar] [CrossRef]
- Martinez-Saguer, I.; Rusicke, E.; Aygören-Pürsün, E.; Heller, C.; Klingebiel, T.; Kreuz, W. Characterization of Acute Hereditary Angioedema Attacks during Pregnancy and Breast-Feeding and Their Treatment with C1 Inhibitor Concentrate. Am. J. Obstet. Gynecol. 2010, 203, 131.e1–131.e7. [Google Scholar] [CrossRef]
- Machado, A.; Pires, R.; Martins, R.; Grumach, A. Pregnancy and Postpartum in Hereditary Angioedema with C1 Inhibitor Deficit in Women Who Have No Access to Therapy. J. Investig. Allergol. Clin. Immunol. 2017, 27, 322–323. [Google Scholar] [CrossRef] [Green Version]
- Montinaro, V.; Castellano, G. Management of Pregnancy and Vaginal Delivery by C1 Inhibitor Concentrate in Two Hereditary Angioedema Twins. Clin. Immunol. 2010, 136, 456–457. [Google Scholar] [CrossRef] [PubMed]
- Hakl, R.; Kuklínek, P.; Krčmová, I.; Králíčková, P.; Freiberger, T.; Janků, P.; Litzman, J.; Litzman, J. Treatment of Hereditary Angioedema Attacks with Icatibant and Recombinant C1 Inhibitor during Pregnancy. J. Clin. Immunol. 2018, 38, 810–815. [Google Scholar] [CrossRef] [PubMed]
- Gorman, P.H. Hereditary Angioedema and Pregnancy: A Successful Outcome Using C1 Esterase Inhibitor Concentrate. Can. Fam. Physician 2008, 54, 365–366. [Google Scholar] [PubMed]
- Winnewisser, J.; Rossi, M.; Späth, P.; Bürgi, H. Type I Hereditary Angio-Oedema. Variability of Clinical Presentation and Course within Two Large Kindreds. J. Intern. Med. 1997, 241, 39–46. [Google Scholar] [CrossRef]
- Caliskaner, Z.; Ozturk, S.; Gulec, M.; Dede, M.; Erel, F.; Karaayvaz, M. A Successful Pregnancy and Uncomplicated Labor with C1INH Concentrate Prophylaxis in a Patient with Hereditary Angioedema. Allergol. Immunopathol. 2007, 35, 117–119. [Google Scholar] [CrossRef]
- Sim, T.C.; Grant, J.A. Hereditary Angioedema: Its Diagnostic and Management Perspectives. Am. J. Med. 1990, 88, 656–664. [Google Scholar] [CrossRef]
- Nathani, F.; Sullivan, H.; Churchill, D. Pregnancy and C1 Esterase Inhibitor Deficiency: A Successful Outcome. Arch. Gynecol. Obstet. 2006, 274, 381–384. [Google Scholar] [CrossRef]
- Cunningham, D.S.; Jensen, J.T. Hereditary angioneurotic edema in the puerperium. A case report. J. Reprod. Med. 1991, 36, 312–313. [Google Scholar]
- Ebert, A.D.; Pritze, W.; Weitzel, H.K. C-1 Esterase Inhibitor Deficiency as an Obstetric Problem: A Case Report. Zentralbl. Gynakol. 1992, 114, 519–522. [Google Scholar]
- Böckers, M.; Bork, K. Kontrazeption und Schwangerschaft beim hereditären Angioödem [Contraception and pregnancy in hereditary angioedema]. Dtsch. Med. Wochenschr. 2008, 112, 507–509. [Google Scholar] [CrossRef]
- Visy, B.; Fust, G.; Varga, L.; Szendei, G.; Takacs, E.; Karadi, I.; Fekete, B.; Harmat, G.; Farkas, H. Sex Hormones in Hereditary Angioneurotic Oedema. Clin Endocrinol 2004, 60, 508–515. [Google Scholar] [CrossRef] [PubMed]
- Bouillet, L.; Gompel, A. Hereditary Angioedema in Women. Allergy Asthma Clin. Immunol. 2013, 33, 505–511. [Google Scholar] [CrossRef] [PubMed]
- Murone, C. Localization of Bradykinin B2 Receptors in the Endometrium and Myometrium of Rat Uterus and the Effects of Estrogen and Progesterone. Endocrinology 1999, 140, 3372–3382. [Google Scholar] [CrossRef] [PubMed]
- Nielsen, E.; Gran, J.T.; Straume, B.; Mellbye, O.J.; Johansen, H.T.; Mollnes, T.E. Hereditary Angio-Oedema: New Clinical Observations and Autoimmune Screening, Complement and Kallikrein-Kinin Analyses. J. Intern. Med. 1996, 239, 119–130. [Google Scholar] [CrossRef]
- Craig, T.; Busse, P.; Gower, R.G.; Johnston, D.T.; Kashkin, J.M.; Li, H.H.; Lumry, W.R.; Riedl, M.A.; Soteres, D. Long-Term Prophylaxis Therapy in Patients with Hereditary Angioedema with C1 Inhibitor Deficiency. Ann. Allergy Asthma Immunol. 2018, 121, 673–679. [Google Scholar] [CrossRef] [Green Version]
- Busse, P.J.; Christiansen, S.C.; Riedl, M.A.; Banerji, A.; Bernstein, J.A.; Castaldo, A.J.; Craig, T.; Davis-Lorton, M.; Frank, M.M.; Li, H.H.; et al. US HAEA Medical Advisory Board 2020 Guidelines for the Management of Hereditary Angioedema. J Allergy Clin Immunol Pract 2021, 9, 132–150. [Google Scholar] [CrossRef]
- Moldovan, D.; Bernstein, J.A.; Hakl, R.; Porebski, G.; Poarch, K.; Lumry, W.R.; Relan, A. Safety of Recombinant Human C1 Esterase Inhibitor for Hereditary Angioedema Attacks during Pregnancy. J. Allergy Clin. Immunol. Pract. 2019, 7, 2938–2940. [Google Scholar] [CrossRef]
- Tranexamic Acid [Package Insert]. Available online: https://www.accessdata.fda.gov/drugsatfda_docs/label/2013/022430s004lbl.pdf (accessed on 19 June 2023).
- Ecallantide [Package Insert]. Available online: https://www.accessdata.fda.gov/drugsatfda_docs/label/2014/125277s071lbl.pdf (accessed on 19 June 2023).
- Lanadelumab [Package Insert]. Available online: https://www.accessdata.fda.gov/drugsatfda_docs/label/2018/761090s000lbl.pdf (accessed on 19 June 2023).
- Greve, J.; Strassen, U.; Gorczyza, M.; Dominas, N.; Frahm, U.-M.; Mühlberg, H.; Wiednig, M.; Zampeli, V.; Magerl, M. Prophylaxis in Hereditary Angioedema (HAE) with C1 Inhibitor Deficiency. JDDG J. Der Dtsch. Dermatol. Ges. 2016, 14, 266–275. [Google Scholar] [CrossRef]
- Cicardi, M.; Bork, K.; Caballero, T.; Craig, T.; Li, H.H.; Longhurst, H.; Reshef, A.; Zuraw, B. Evidence-Based Recommendations for the Therapeutic Management of Angioedema Owing to Hereditary C1 Inhibitor Deficiency: Consensus Report of an International Working Group. Allergy 2011, 67, 147–157. [Google Scholar] [CrossRef]
- Craig, T.J.; Bewtra, A.K.; Bahna, S.L.; Hurewitz, D.; Schneider, L.C.; Levy, R.J.; Moy, J.N.; Offenberger, J.; Jacobson, K.W.; Yang, W.H.; et al. C1 Esterase Inhibitor Concentrate in Hereditary Angioedema Attacks--Final Results of the I.M.P.A.C.T. Study. Allergy 2011, 66, 1604–1611. [Google Scholar] [CrossRef]
- Yakaboski, E.; Motazedi, T.; Banerji, A. Hereditary Angioedema: Special Considerations in Women. Allergy Asthma Proc. 2020, 41, S47–S50. [Google Scholar] [CrossRef] [PubMed]
- Williams, A.H.; Craig, T.J. Perioperative Management for Patients with Hereditary Angioedema. Allergy Rhinol 2015, 6, 50–55. [Google Scholar] [CrossRef] [PubMed]
- Gandhi, P.K.; Gentry, W.A.; Bottorff, M.B. Thrombotic Events Associated with C1 Esterase Inhibitor Products in Patients with Hereditary Angioedema: Investigation from the United States Food and Drug Administration Adverse Event Reporting System Database. Pharmacotherapy 2012, 32, 902–909. [Google Scholar] [CrossRef] [PubMed]
- Wentzel, N.; Panieri, A.; Ayazi, M.; Ntshalintshali, S.D.; Pourpak, Z.; Hawarden, D.; Potter, P.; Levin, M.E.; Fazlollahi, M.R.; Peter, J. Fresh Frozen Plasma for On-Demand Hereditary Angioedema Treatment in South Africa and Iran. World Allergy Organ. J. 2019, 12, 100049. [Google Scholar] [CrossRef] [PubMed] [Green Version]
- Valerieva, A.; Staevska, M.; Jesenak, M.; Hrubiskova, K.; Sobotkova, M.; Zachova, R.; Hakl, R.; Andrejevic, S.; Suiter, T.; Grivcheva-Panovska, V.; et al. Recombinant Human C1 Esterase Inhibitor as Short-Term Prophylaxis in Patients with Hereditary Angioedema. J. Allergy Clin. Immunol. Pract. 2020, 8, 799–802. [Google Scholar] [CrossRef] [PubMed]
- Craig, T.J.; Zuraw, B.L.; Longhurst, H.; Cicardi, M.; Bork, K.; Grattan, C.; Katelaris, C.H.; Sussman, G.; Keith, P.K.; Yang, W.H.; et al. Long-Term Outcomes with Subcutaneous C1-Inhibitor Replacement Therapy for Prevention of Hereditary Angioedema Attacks. J. Allergy Clin. Immunol. Pract. 2019, 7, 1793–1802. [Google Scholar] [CrossRef]
- Farkas, H.; Kőhalmi, K.V.; Veszeli, N.; Tóth, F.; Varga, L. First Report of Icatibant Treatment in a Pregnant Patient with Hereditary Angioedema. J. Obstet. Gynaecol. Res. 2016, 42, 1026–1028. [Google Scholar] [CrossRef]
- Kaminsky, L.W.; Kelbel, T.; Ansary, F.; Craig, T. Multiple Doses of Icatibant Used during Pregnancy. Allergy Rhinol. 2017, 8, ar-2017. [Google Scholar] [CrossRef] [Green Version]
- Šimac, D.V.; Štimac, T.; Novak, S. Is Icatibant Safe for the Treatment of Hereditary Angioedema during Pregnancy? Curr. Allergy Asthma Rep. 2022, 22, 135–140. [Google Scholar] [CrossRef]
- Jindal, A.K.; Sil, A.; Aggarwal, R.; Vinay, K.; Bishnoi, A.; Suri, D.; Rawat, A.; Kumaran, M.S.; Saikia, B.; Sarkar, R.; et al. Management of hereditary angioedema in resource-constrained settings: A consensus statement from Indian subcontinent. Asia Pac. Allergy 2023, 13, 60–65. [Google Scholar] [CrossRef]
- Liu, S.; Xu, Y.; Liu, Y.; Zhi, Y. Hereditary angioedema: A Chinese perspective. Eur. J. Dermatol. 2019, 29, 14–20. [Google Scholar] [CrossRef] [PubMed]
- Banerji, A.; Anderson, J.; Johnston, D.T. Optimal Management of Hereditary Angioedema: Shared Decision-Making. J. Asthma Allergy 2021, 14, 119–125. [Google Scholar] [CrossRef] [PubMed]
Pre-Operative Period: | Peri-Operative Period: | Post-Operative Period: |
---|---|---|
Ensure availability of at least 2 doses of appropriate rescue medication * | Use local anesthetic and/or nerve blocking to avoid need for intubation | Ensure availability of appropriate rescue medication throughout recovery and discharge periods |
Assess for history of prior HAE attacks during surgical procedures | Perform vaginal delivery when possible to avoid surgical trauma | Prior to discharge, provide patient with education on rescue medication self-administration, if needed |
Pretreat patient with appropriate STP medication * | Maintain ability to protect airway at all times during procedure |
Drug Name (Route of Administration) | Rescue Therapy | Short-Term Prophylaxis | Long-Term Prophylaxis | Dosage |
---|---|---|---|---|
pdC1-INH (IV) | 1st line | 1st line | 1st line | Rescue: 20 IU/kg STP: 20 IU/kg 1–12 h before stressor LTP: 1000 IU every 3–4 days (may be increased up to 2500 IU) |
pdC1-INH (SC) | - | - | 1st line | 60 IU/kg twice weekly |
Fresh frozen plasma (IV) | 2nd line | 2nd line | - | 2 to 4 units |
Recombinant C1-INH (IV) | 2nd line | 2nd line | - | 50 IU/kg up to 4200 IU |
Tranexamic acid (oral) | 3rd line | 3rd line | - | 20–40 mg/kg |
Berotralstat (oral) | Recently approved for LTP. Insufficient data for use in pregnancy and lactation | 150 mg daily | ||
Icatibant (SC) | Approved for rescue therapy. Insufficient data for use in pregnancy and lactation | 30 mg | ||
Lanadelumab (SC) | Approved for LTP. Insufficient data for use in pregnancy and lactation | 300 mg every 2–4 weeks | ||
Androgens (oral) | Contraindicated during pregnancy and lactation | - | ||
Ecallantide (SC) | Contraindicated during pregnancy and lactation | - |
Consider long-term prophylaxis for most pregnant women who have active HAE symptoms |
Pretreat with 20 IU/kg intravenous pdC1-INH 1 h before procedures, dental work, or surgery. Recombinant C1-INH and fresh frozen plasma may be alternate therapies if pdC1-INH is not available |
Have 2 doses of rescue therapy available of C1-INH or recombinant C1-INH at all times |
During delivery, use vaginal delivery when possible |
Always have capability to protect the airway during procedures and for 72 h after procedures |
Consider long-term prophylaxis for most pregnant women who have active HAE symptoms |
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Park, K.; Yeich, A.; Craig, T. Preferred Therapy for Patients with Hereditary Angioedema during Pregnancy. Future Pharmacol. 2023, 3, 586-596. https://doi.org/10.3390/futurepharmacol3030037
Park K, Yeich A, Craig T. Preferred Therapy for Patients with Hereditary Angioedema during Pregnancy. Future Pharmacology. 2023; 3(3):586-596. https://doi.org/10.3390/futurepharmacol3030037
Chicago/Turabian StylePark, Kristen, Andrew Yeich, and Timothy Craig. 2023. "Preferred Therapy for Patients with Hereditary Angioedema during Pregnancy" Future Pharmacology 3, no. 3: 586-596. https://doi.org/10.3390/futurepharmacol3030037
APA StylePark, K., Yeich, A., & Craig, T. (2023). Preferred Therapy for Patients with Hereditary Angioedema during Pregnancy. Future Pharmacology, 3(3), 586-596. https://doi.org/10.3390/futurepharmacol3030037