Acil Serviste Herediter Anjioödem’e Yaklaşım

Yıl 2020, , 152 - 161, 16.03.2020

Öner Özdemir

https://doi.org/10.31832/smj.627591

Cited By: 1

Öz

Herediter anjioödem
(HAÖ) nadir görülen, genelde C1 inhibitör (C1-INH) eksikliğine bağlı,
tekrarlayan anjioödem ataklarıyla bilinen ölümcül olabilen otozomla dominant
geçişli bir hastalıktır. Anjioödem atakları aniden oluşur, sıklıkla tahmin
edilemez ve tipik olarak ekstremite, gövde, yüz, karın, genital organlar ve üst
solunum yollarını tutan şişliklere yol açar. Herediter anjioödem hastalarında
tipik olarak eşlik eden ürtiker olmaması sıklıkla nonhistaminerjik (bradikinerjik)
anjioödem şeklinde düşünülmesine yol açar. Ataklar önemli derecede işlevsel
bozukluğa, yaşam kalitesinin azalmasına ve larengeal atak olgularında ise ölüme
yol açabilir. Herediter anjioödemin karın tutulumuyla giden atağı genelde tanıda
gecikme ve uygunsuz tedaviye neden olan ailevi Akdeniz ateşi (FMF) ve apandisit
gibi bazı akut batın durumlarını taklit eder. Kompleksliğinden dolayı HAÖ
hastalarının bakımı bazen zorlayıcı olabilir. Herediter anjioödem tedavisi kısa
ve uzun süreli profilaksi ile atak tedavilerini içerir. Acilde HAÖ bakımı
yanlış tanı ve sınırlı sayıda tedavi seçeneği ile gecikebilir. Son iki dekattır
HAÖ atak tedavisinde bazı tedavi seçenekleri mevcuttur. Bu seçeneklerin çoğu
ABD, Avrupa, Birleşik Krallık ve Avustralya’da yaşayan HAÖ hastaları için
günümüzde ulaşılabilir durumda olmasına rağmen, gelişmekte olan ülkelerde
sınırlı sayıda tercihler hazırda bulunur. Dört tür akut/atak tedavi seçenekleri
arasında, plazma kaynaklı C1-INH konsantresi, rekombinant C1-INH konsantresi,
ecallantide ve ikatibant günümüzde kullanılanlardır.

Anahtar Kelimeler

Herediter anjioödem, atak, acil servis

Destekleyen Kurum

Yok

Proje Numarası

Yok

Teşekkür

Yok

Kaynakça

• 1- Özdemir Ö. Herediter anjioödemde tanı ve ayırıcı tanı. Turkiye Klinikleri J Allergy-Special Topics 2011;4(2):21-35.
• 2- Kelly M, Donnelly JP, McAnnally JR, Wang HE. National estimates of emergency department visits for angioedema and allergic reactions in the United States. Allergy Asthma Proc. 2013;34:150–4.
• 3- Bertazzoni G, Spina MT, Scarpellini MG, Buccelletti F, De Simone M, Gregori M, et al. Drug-induced angioedema: experience of Italian emergency departments. Intern Emerg Med. 2014;9:455–62.
• 4- Felder S, Curtis RM, Ball I, Borici-Mazi R. Prognostic factors in outcome of angioedema in the emergency department. Allergy Asthma Proc. 2014; 35(5):362-70.
• 5- Bernstein JA, Cremonesi P, Hoffmann TK, Hollingsworth J. Angioedema in the emergency department: a practical guide to differential diagnosis and management. Int J Emerg Med. 2017; 10(1):15.
• 6- Bowen T, Cicardi M, Farkas H, Bork K, Longhurst HJ, Zuraw B, et al. 2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema. Allergy Asthma Clin Immunol. 2010; 6(1):24.
• 7- Özdemir Ö, Elmas B. Türkiye’de bir şehirde indeks herediter anjioödem hastalarının uzak ve yakın test edilmemiş akrabalarında herediter anjioödem prevalans ve klinik belirtileri. JAREM 2017; 7:51-7.
• 8- Zuraw BL. Hereditary angioedema with normal C1 inhibitor: Four types and counting. J Allergy Clin Immunol. 2018; 141(3):884-5.
• 9- Lumry WR. Overview of epidemiology, pathophysiology, and disease progression in hereditary angioedema. Am J Manag Care. 2013; 19(7 Suppl):s103-10.
• 10- Gülbahar O, Gelincik A, Sin A, Güleç M, Yılmaz M, Gökmen NM, et al. Herediter anjioödem. Asthma Allergy Immunol 2010; 8: 125–38.
• 11- Zuraw BL, Christiansen SC. HAE pathophysiology and underlying mechanisms. Clin Rev Allergy Immunol. 2016;51(2):216-29.
• 12- Caccia S, Suffritti C, Cicardi M. Pathophysiology of hereditary angioedema. Pediatr Allergy Immunol Pulmonol. 2014; 27(4):159-163.
• 13- Wu MA, Perego F, Zanichelli A, Cicardi M. Angioedema phenotypes: disease expression and classification. Clin Rev Allergy Immunol. 2016; 51(2):162-9.
• 14- Dewald G, Bork K. Missense mutations in the coagulation factor XII (Hageman factor) gene in hereditary angioedema with normal C1 inhibitor. Biochem Biophys Res Commun 2006; 343:1286-9.
• 15- Bork K, Wulff K, Steinmüller-Magin L, Braenne I, Staubach-Renz P, Witzke G, et al. Hereditary angioedema with a mutation in the plasminogen gene. Allergy 2018; 73(2):442-50.
• 16- Bafunno V, Firinu D, D'Apolito M, Cordisco G, Loffredo S, Leccese A, et al. Mutation of the angiopoietin-1 gene (ANGPT1) associates with a new type of hereditary angioedema. J Allergy Clin Immunol. 2018; 141(3): 1009-17.
• 17- Cicardi M, Aberer W, Banerji A, Bas M, Bernstein JA, Bork K, et al. Classification, diagnosis, and approach to treatment for angioedema: consensus report from the Hereditary Angioedema International Working Group. Allergy 2014; 69(5):602-16.
• 18- Moellman JJ, Bernstein JA, Lindsell C, Banerji A, Busse PJ, Camargo CA Jr, et al. A consensus parameter for the evaluation and management of angioedema in the emergency department. Acad Emerg Med. 2014; 21(4):469-84. 19- Maurer M, Magerl M, Ansotegui I, Aygören-Pürsün E, Betschel S, Bork K, et al. The international WAO/EAACI guideline for the management of hereditary angioedema-The 2017 revision and update. Allergy 2018; 73(8):1575-96.
• 20- Betschel S, Badiou J, Binkley K, Hébert J, Kanani A, Keith P, et al. Canadian hereditary angioedema guideline. Allergy Asthma Clin Immunol. 2014;10(1):50.
• 21- Özdemir Ö . Mast Hücre- Aracılı (Histaminerjik) Anjioödeme Yaklaşım: Klinik Spektrum ve Tanı. Sakarya Tıp Dergisi. 2019; 9(3): 378-369.
• 22- Busse PJ, Smith T. Histaminergic Angioedema. Immunol Allergy Clin North Am. 2017; 37(3):467-81.
• 23- Craig TJ, Bernstein JA, Farkas H, Bouillet L, Boccon-Gibod I. Diagnosis and treatment of bradykinin-mediated angioedema: outcomes from an angioedema expert consensus meeting. Int Arch Allergy Immunol. 2014;165(2):119-27.
• 24- Li HH. Pearls and pitfalls in the diagnosis of hereditary angioedema. Allergy Asthma Proc. 2019; 40(4):282-4.
• 25- Betschel S, Avilla E, Kanani A, Kastner M, Keith P, Binkley K, et al. Development of the Hereditary Angioedema Rapid Triage Tool (HAE-RT). J Allergy Clin Immunol Pract. 2019 Jun 22. pii: S2213-2198(19)30562-8. [Epub ahead of print]
• 26- Craig T, Aygören Pürsün E, Bork K, Bowen T, Boysen H, Farkas H, et al. WAO guideline for the management of hereditary angioedema. World Allergy Organ J 2012; 5(12): 182–99.
• 27- Farkas H, Martinez‐Saguer I, Bork K, Bowen T, Craig T, Frank M, et al. International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency. Allergy. 2017; 72(2): 300–13.
• 28- Frank MM, Zuraw B, Banerji A, Bernstein JA, Craig T, Busse P, et al. Management of children with hereditary angioedema due to C1 inhibitor deficiency. Pediatrics 2016; 138(5). pii: e20160575.
• 29- Nasr IH, Manson AL, Al Wahshi HA, Longhurst HJ. Optimizing hereditary angioedema management through tailored treatment approaches. Expert Rev Clin Immunol. 2016; 12(1): 19-31.
• 30- Sabharwal G, Craig T. Pediatric hereditary angioedema: an update. F1000Res. 2017; 6.
• 31- Pattanaik D, Lieberman JA. Pediatric angioedema. Curr Allergy Asthma Rep. 2017; 17(9): 60.
• 32- MacGinnitie AJ. Pediatric hereditary angioedema. Pediatr Allergy Immunol 2014: 25: 420–7.
• 33- Lang DM, Aberer W, Bernstein JA, Chng HH, Grumach AS, Hide M, et al. International consensus on hereditary and acquired angioedema. Ann Allergy Asthma Immunol. 2012; 109 (6): 395-402.
• 34- Zuraw BL, Bernstein JA, Lang DM, Craig T, Dreyfus D, Hsieh F, et al. A focused parameter update: hereditary angioedema, acquired C1inhibitor deficiency, and angiotensin-converting enzyme inhibitor-associated angioedema. J Allergy Clin Immunol. 2013; 131(6): 1491-3.
• 35- Hakl R, Kuklínek P, Krčmová I, Králíčková P, Freiberger T, Janků P, et al. Treatment of Hereditary Angioedema Attacks with Icatibant and Recombinant C1 Inhibitor During Pregnancy. J Clin Immunol. 2018;38(7):810-5.
• 36- Jose J, Zacharias J, Craig T. Review of select practice parameters, evidence-based treatment algorithms, and international guidelines for hereditary angioedema. Clin Rev Allergy Immunol. 2016; 51(2):193-206.
• 37- Longhurst H. Optimum Use of Acute Treatments for Hereditary Angioedema: Evidence-Based Expert Consensus. Front Med (Lausanne). 2018; 4:245.
• 38- Tse KY, Zuraw BL, Chen Q, Christiansen SC. Anabolic androgen use in the management of hereditary angioedema: Not so cheap after all. Ann Allergy Asthma Immunol. 2017; 118 (4):456-60.e1.
• 39- Bernstein JA, Relan A, Harper JR, Riedl M. Sustained response of recombinant human C1 esterase inhibitor for acute treatment of hereditary angioedema attacks. Ann Allergy Asthma Immunol 2017; 118(4):452-5.
• 40- Baker JW, Reshef A, Moldovan D, Harper JR, Relan A, Riedl MA. Recombinant Human C1-esterase inhibitor to treat acute hereditary angioedema attacks in adolescents. J Allergy Clin Immunol Pract. 2017; 5(4):1091-7.
• 41- Riedl MA, Li HH, Cicardi M, Harper JR, Relan A. Recombinant human C1 esterase inhibitor for acute hereditaryangioedema attacks with upper airway involvement. Allergy Asthma Proc. 2017; 38(6):462-6.
• 42- Chen M, Riedl MA. Emerging therapies in hereditary angioedema. Immunol Allergy Clin North Am. 2017; 37(3):585-95.
• 43- Lumry WR. Current and emerging therapies to prevent hereditary angioedema attacks. Am J Manag Care. 2018; 24(14 Suppl):S299-S307.
• 44- Perego F, Wu MA, Valerieva A, Caccia S, Suffritti C, Zanichelli A, et al. Current and emerging biologics for the treatment of hereditary angioedema. Expert Opin Biol Ther. 2019: 1-10.
• 45- Farkas H. Hereditary angioedema: examining the landscape of therapies and preclinical therapeutic targets. Expert Opin Ther Targets. 2019; 23(6):457-9.
• 46- Baysan A, Güleç M. Herediter anjioödemde atakların önlenmesi. Turkiye Klinikleri J Allergy- Special Topics 2011;4(2): 36-43.
• 47- Akkor Gelincik A. Herediter anjioödemde akut atakların tedavisi. Turkiye Klinikleri J Allergy- Special Topics 2011;4(2):44-50.
• 48- Duffey H, Firszt R. Management of acute attacks of hereditary angioedema: role of ecallantide. J Blood Med. 2015; 6:115-23.
• 49- Katelaris CH. Acute management of hereditary angioedema attacks. Immunol Allergy Clin North Am. 2017; 37(3):541-56.
• 50- Otani IM, Christiansen SC, Busse P, Camargo CA Jr, Zuraw BL, Riedl MA, et al. Emergency department management of hereditary angioedema attacks: Patient perspectives. J Allergy Clin Immunol Pract. 2017; 5(1):128-34.e4.
• 51- Zanichelli A, Azin GM, Cristina F, Vacchini R, Caballero T. Safety, effectiveness, and impact on quality of life of self-administration with plasma-derived nanofiltered C1 inhibitor (Berinert®) in patients with hereditary angioedema: the SABHA study. Orphanet J Rare Dis 2018; 13(1):51.
• 52- Li H, Riedl M, Kashkin J. Update on the use of C1-esterase inhibitor replacement therapy in the acute and prophylactic treatment of hereditary angioedema. Clin Rev Allergy Immunol 2019; 56(2):207-18.
• 53- Caballero T, Aberer W, Longhurst HJ, Maurer M, Zanichelli A, Perrin A, et al. The Icatibant Outcome Survey: experience of hereditary angioedema management from six European countries. J Eur Acad Dermatol Venereol. 2017; 31(7):1214-22.
• 54- Longhurst HJ, Zanichelli A, Caballero T, Bouillet L, Aberer W, Maurer M, et al. Comparing acquired angioedema with hereditary angioedema (types I/II): findings from the Icatibant Outcome Survey. Clin Exp Immunol. 2017; 188(1):148-53.
• 55- Lumry WR, Farkas H, Moldovan D, Toubi E, Baptista J, Craig T, et al. Icatibant for multiple hereditary angioedema attacks across the controlled and open-label extension phases of FAST-3. Int Arch Allergy Immunol. 2015;168(1):44-55.
• 56- Farkas H, Reshef A, Aberer W, Caballero T, McCarthy L, Hao J, et al. Treatment effect and safety of Icatibant in pediatric patients with hereditary angioedema. J Allergy Clin Immunol Pract. 2017; 5(6):1671-8.e2.
• 57- Longhurst HJ, Aberer W, Bouillet L, Caballero T, Maurer M, Fabien V, et al. The Icatibant Outcome Survey: treatment of laryngeal angioedema attacks. Eur J Emerg Med 2016; 23(3):224-7.
• 58- Craig TJ, Li HH, Riedl M, Bernstein JA, Lumry WR, MacGinnitie AJ, et al. Characterization of anaphylaxis after ecallantide treatment of hereditary angioedema attacks. J Allergy Clin Immunol Pract. 2015;3(2):206-12.e4.
• 59- Bowen T, Cicardi M, Farkas H, Bork K, Kreuz W, Zingale L, et al. Canadian 2003 international consensus algorithm for the diagnosis, therapy, and management of hereditary angioedema. J Allergy Clin Immunol. 2004; 114(3):629-37.