Abstract
Abstract
Angioedema is described as local, restricted noninflammatory edema as a result of augmented plasma leakage fromthe capillaries situated in the profound layers of the skin andthe mucosae. The major mediators such as histamine and bradykinin play a role in the pathophysiology of most of the angioedema cases. According to these mediators, angioedema is essentially classified as histaminergic or bradykininergic. Angioedema can be observed with wheals as anindicator of urticaria, and this type is often associated withhistaminergic (allergic, IgE-mediated) type. Histaminergicangioedema is the most common type of angioedema. Angioedema is categorized into either acute (symptoms lasting less than 6 weeks) or chronic (lasting more than 6 weeks) form. It is further characterized into angioedema manifesting with or without wheals. Angioedema with wheals may be acute, chronic, spontaneous or inducible. Angioedema without wheals (AEWOW) is still seen in approximately 10% of urticaria patients, but it can also take place as a discrete entity. In 2014 Hereditary Angioedema International Working Group classification, AEWOW is mainly categorized into hereditary and acquired types. Hereditary AEWOW forms are as follows: Hereditary angioedema (HAE) with genetic C1 inhibitor (C1-INH) deficiency,HAE with normal C1-INH but with Factor XII gene mutation, and unknown HAE; acquired forms of AEWOW have4 subtypes. Acquired C1-INH deficiency, angiotensin converting enzyme inhibitor (ACE-i) induced, idiopathic histaminergic and idiopathic non-histaminergic angioedema. From these types, just idiopathic histaminergic is histaminergic, the others are bradykininergic angioedemas. Hereditary angioedema is a rare congenital disease mainly attributable to the SERPING1 gene mutations, programmingthe C1-INH protein that yields to plasma deficiency, resulting in recurrent attacks of severe angioedema. As of 2018,over 490 different mutations were demonstrated in the region of C1-INH gene (SERPINGI). It is now known thatC1-INH deficiency excites the plasma contact (kallikreinkinin) system, which ultimately results in bradykininover production. After the year 2000, the most recent development in the field of HAE, 3 new types of HAE with “normal” C1-INH were reported. Several abnormalities in thegenes of Factor XII, Angiopoietin-1 and Plasminogen havebeen identified in this novel disease entity. Due to obviousin consistency of clinical pictures, complete understanding of recurrent angioedema phenotypes and the underlying mediator of symptoms are essential for precise diagnosis andselecting the appropriate therapy options. Although the main therapies of histaminergic angioedema are antihistamines,corticosteroids, and epinephrine, these medications will notwork in especially hereditary angioedema and some typesof acquired angioedema. In this present review, the distinctive epidemiology, pathophysiology and clinical spectrumof histaminergic and bradykininergic angioedema, and differential diagnosis are discussed under the light of recent literature, along with our case scenarios.
Keywords
histaminergic angioedema urticaria hereditary angioedema C1 inhibitor factorXII angiopoietin-1 plasminogen
References
- Kaynaklar 1- Misra L, Khurmi N, Trentman TL. Angioedema: Classification,management and emerging therapies for the perioperativephysician. Indian J Anaesth. 2016; 60(8):534-41. 2- Kaplan AP, Greaves MW. Angioedema. J Am Acad Dermatol.2005; 53(3):373-88. 3- Wu MA, Perego F, Zanichelli A, Cicardi M. Angioedema phe-notypes: disease expression and classification. Clin Rev AllergyImmunol 2016; 51(2):162-9. 4- Pattanaik D, Lieberman JA. Pediatric angioedema. Curr Al-lergy Asthma Rep. 2017; 17(9):60. 5- Cicardi M, Suffritti C, Perego F, Caccia S. Novelties in the diag-nosis and treatment of angioedema. J Investig Allergol Clin Im-munol 2016; 26 (4): 212 -21. 6- Jaiganesh T, Wiese M, Hollingsworth J, Hughan C, Kamara M,Wood P, et al. Acute angioedema: recognition and management inthe emergency department. Eur J Emerg Med. 2013; 20(1):10-7. 7- Rye Rasmussen EH, Bindslev-Jensen C, Bygum A. Angioedema--assessment and treatment. Tidsskr Nor Laegeforen. 2012;132(21): 2391-5. 8- Gill P, Betschel SD. The clinical evaluation of angioedema. Immunol Allergy Clin North Am. 2017; 37(3):449-66. 9- LoVerde D, Files DC, Krishnaswamy G. Angioedema. Crit CareMed. 2017; 45(4):725-35. 10- Triggianese P, Guarino MD, Pellicano C, Borzi M, Greco E,Modica S, et al. Recurrent angioedema: Occurrence, features,and concomitant diseases in an İtalian single-center study. IntArch Allergy Immunol. 2017; 172(1):55-63. 11- Bouillet L, Boccon-Gibod I, Berard F, Nicolas JF. Recurrentangioedema: diagnosis strategy and biological aspects. Eur JDermatol 2014; 24(3):293-6. 12- Lewis LM. Angioedema: etiology, pathophysiology, currentand emerging therapies. J Emerg Med. 2013; 45(5):789-96. 13-James C, Bernstein JA. Current and future therapies for the tre-atment of histamine-induced angioedema. Expert Opin Phar-macother 2017; 18(3):253-62. 14- Bernstein JA, Lang DM, Khan DA, et al: The diagnosis andmanagement of acute and chronic urticaria: 2014 update. J Al-lergy Clin Immunol 2014;133:1270-7 15- Powell RJ, Leech SC, Till S,Huber PAJ, Nasser SM, ClarkAT.BSACI guideline for the management of chronic urticaria andangioedema. Clin Exp Allergy. 2015; 45:547–65. 16- Maurer M, Magerl M, Ansotegui I, Aygören-Pürsün E, Betsc-hel S, Bork K, et al. The international WAO/EAACI guidelinefor the management of hereditary angioedema-The 2017 revisi-on and update. Allergy 2018; 73(8):1575-96. 17- Huston DP, Sabato V. Decoding the enigma of urticaria andangioedema. J Allergy Clin Immunol Pract. 2018; 6(4):1171-5. 18- Giavina-Bianchi P, Aun MV, Motta AA, Kalil J, Castells M.Classification of angioedema by endotypes. Clin Exp Allergy2015; 45(6):1142-3. 19- Bas M, Adams V, Suvorava T, Niehues T, Hoffmann TK, KojdaG. Nonallergic angioedema: role of bradykinin. Allergy. 2007;62(8):842-56. 20- Busse PJ, Smith T. Histaminergic Angioedema. Immunol Al-lergy Clin North Am. 2017; 37(3):467-81. 21- Moellman JJ, Bernstein JA, Lindsell C, Banerji A, Busse PJ,Camargo CA Jr, et al. A consensus parameter for the evaluati-on and management of angioedema in the emergency depart-ment. Acad Emerg Med. 2014; 21(4):469-84. 22- Barbara DW, Ronan KP, Maddox DE, Warner MA. Periopera-tive angioedema: background, diagnosis, and management. JClin Anesth 2013; 25(4):335-43. 23-Zuberbier T, Bernstein JA. A Comparison of the United Statesand international perspective on chronic urticaria guidelines. JAllergy Clin Immunol Pract. 2018; 6(4):1144-51. 24- Cicardi M, Aberer W, Banerji A, Bas M, Bernstein JA, Bork K,et al. Classification, diagnosis, and approach to treatment forangioedema: consensus report from the Hereditary Angioede-ma International Working Group. Allergy 2014; 69(5):602-16 25- James C, Bernstein JA. Current and future therapies for thetreatment of histamine-induced angioedema. Expert OpinPharmacother 2017; 18(3):253-62. 26- Mansi M, Zanichelli A, Coerezza A, Suffritti C, Wu MA, Vacchi-ni R, et al. Presentation, diagnosis and treatment of angioede-ma without wheals: a retrospective analysis of a cohort of 1058patients. J Intern Med. 2015; 277(5):585-93. 27-Malbrán E, Fernández Romero D, Juri MC, Larrauri BJ,Malbrán A. Epidemiology of angioedema without wheals in anallergy and immunology center. Medicina (B Aires). 2015;75(5):273-6. 28-Faisant C, Boccon-Gibod I, Mansard C, Dumestre Perard C,Pralong P, Chatain C, et al. Idiopathic histaminergic angioede-ma without wheals: a case series of 31 patients. Clin Exp Im-munol. 2016; 185(1): 81-5. 29-Eli M, Joseph M, Kuznik B, Menachem S. Chronic idiopathicangioedema: a single center experience. Int J Dermatol 2014;53(10):e421-7. 30-Bucher MC, Petkovic T, Helbling A, Steiner UC. Idiopathic non-his-taminergic acquired angioedema: a case series and discussion ofpublished clinical trials. Clin Transl Allergy. 2017;7:27. 31-Hahn J, Hoffmann TK, Bock B, Nordmann-Kleiner M, Trainotti S,Greve J. Angioedema. Dtsch Arztebl Int. 2017; 114(29-30):489-96. 32- Bork K, Wulff K, Witzke G, Hardt J. Hereditary angioedemawith normal C1-INH with versus without specific F12 genemutations. Allergy 2015;70(8):1004-12. 33- Lumry WR. Overview of epidemiology, pathophysiology, anddisease progression in hereditary angioedema. Am J ManagCare 2013; 19(7 Suppl):s103-10. 34- Cugno M, Zanichelli A, Foieni F, Caccia S, Cicardi M. C1-in-hibitor deficiency and angioedema: molecular mechanismsand clinical progress. Trends Mol Med. 2009;15(2):69-78. 35- Zuraw BL, Christiansen SC. HAE pathophysiology and underl-ying mechanisms. Clin Rev Allergy Immunol. 2016; 51(2):216-29. 36- Caccia S, Suffritti C, Cicardi M. Pathophysiology of hereditary angi-oedema. Pediatr Allergy Immunol Pulmonol. 2014; 27(4):159-63. 37- Zuraw BL. Hereditary angioedema with normal C1 inhibitor: Fourtypes and counting. J Allergy Clin Immunol. 2018; 141(3):884-5. 38- Zeerleder S, Levi M. Hereditary and acquired C1-inhibitor-de-pendent angioedema: from pathophysiology to treatment. AnnMed. 2016; 48(4):256-67. 39- Kaplan AP. Enzymatic pathways in the pathogenesis of he-reditary angioedema: the role of C1 inhibitor therapy. J AllergyClin Immunol. 2010; 126(5):918-25. 40- Schmaier AH. The contact activation and kallikrein/kininsystems: pathophysiologic and physiologic activities. J ThrombHaemost. 2016; 14(1):28-39. 41- Charlesworth EN. Differential diagnosis of angioedema. Al-lergy Asthma Proc. 2002; 23(5):337-9. 42- Kaplan AP, Joseph K. The bradykinin-forming cascade and itsrole in hereditary angioedema. Ann Allergy Asthma Immunol.2010; 104(3):193-204. 43- Cicardi M, Zuraw BL. Angioedema due to bradykinin dysregu-lation. J Allergy Clin Immunol Pract. 2018; 6(4):1132-41. 44-Cicardi M, Zanichelli A. Diagnosing angioedema. Immunol Al-lergy Clin North Am. 2013; 33(4): 449-56. 45-Bohra S, Kariya PB, Bargale SD, Kiran S. Clinicopathologicalsignificance of Melkersson-Rosenthal syndrome. BMJ CaseRep. 2015 Jul 31;2015. pii: bcr2015210138. 46- Sher J, Davis-Lorton M. Angioedema with normal laboratory values:the next step. Curr Allergy Asthma Rep 2013; 13(5):563-70.
Abstract
Öz
Anjiyoödem mukoza ve cildin derin tabakalarında bulunan kapillerlerden artmış plaz-ma sızmasına bağlı lokal, enflamatuar olmayan ve kendini sınırlayan bir ödem olaraktanımladır. Histamin ve bradikinin gibi iki ana mediatör, anjiyoödem vakalarının çoğu-nun patofizyolojisinde rol oynarlar. Bu mediatörlere göre, anjiyoödem esas olarak his-tamin- ve bradikinin-aracılı hastalık olarak sınıflandırılır. Anjiyoödem ürtikerin bir be-lirtisi olarak kabarıklıkla beraber olabilir ve bu şekli çoğunlukla histaminerjik (allerjik,IgE-aracılı) tiple ilişkilidir. Histaminerjik anjiyoödem en sık anjiyoödem tipidir. Anji-yoödem, akut (semptomlar 6 haftadan kısa sürerse) veya kronik (semptomlar 6 hafta-dan uzun sürerse) olarak iki şekilde ayrılabilir. Anjiyoödem, ürtikerle (kaşıntılı kaba-rıklık) beraber olup olmamasına göre de daha ileri sınıflanabilir. Kaşıntılı kabarıkla be-raber olan anjiyoödem, akut ya da kronik, spontan ya da indüklenebilir bir ürtikerin gös-tergesidir. Kabarıkla olmayan anjiyoödem, ürtikerli hastaların yine de %10’un da gö-rülebilir fakat ayrı bir semptom olarak da oluşabilir. Kabarıklık oluşmayan (kabarcık-sız) anjiyoödemin herediter şekilleri şöyledir: genetik C1 inhibitor (C1-INH) eksikli-ğiyle giden herediter anjiyoödem (HAÖ), faktör XII gen mutasyonu olup normal C1-INH ile giden HAÖ ve nedeni bilinmeyen HAÖ. Kabarıklık oluşmayan anjiyoödeminakkiz şekilleri 4 alttiptir. Akkiz C1-INH eksikliği, anjiyotensin konverting enzim inhi-bitor (ACE-i)’ün indüklediği, idiyopatik histaminerjik ve idiyopatik histaminerjik ol-mayan anjiyoödem. Bu tiplerden sadece idiyopatik histaminerjik olan histamin aracı-lı, diğerleri ise bradikinin aracılığıyla oluşan anjiyoödemlerdir. Herediter anjiyoödem,C1-INH kodlayan SERPING1 genindeki mutasyonlar sonucunda plazma düzeyinde düş-meye bağlı tekrarlayan ciddi şişme (anjiyoödem) ataklarıyla seyreden nadir görülen ge-netik bir bozukluktur. 2018 itibarıyla, C1-INH (SERPINGI) geninde 490’den fazla de-ğişik mutasyon bildirilmiştir. Günümüzde C1-INH eksikliğinin plazma kontakt (kallik-rein-kinin) sisteminin aktivasyonuna ve nihai olarak bradikinin aşırı üretimine yol aç-tığı bilinmektedir. 2000 yılı sonrasında, HAÖ hastalığı hakkındaki en yeni gelişme, C1-INH’in düzeyinin “normal” olduğu 3 yeni HAÖ tipinin bildirilmesidir. Faktör XII, An-jiyopoietin-1 ve Plazminojen gibi birkaç gende bazı anomaliler hastalığın bu yeni tipin-de tanımlanmıştır. Doğru teşhis ve uygun tedaviyi seçmek için, klinik tablonun bariz he-terojenliğinden dolayı, anjiyoödem fenotipinin ve semptomların altında yatan medya-törün iyi bilinmesi esastır. Histamin-aracılı anjiyoödemin asıl tedavisi antihistaminik,kortikosteroit ve epinefrin olmakla beraber, bu ilaçlar herediter ve bazı akkiz anjiyoö-dem tiplerinde işe yaramayacaktır. Bu derlemede, histaminerjik ve bradikinerjik anji-yoödeme özgün epidemiyoloji, patofizyoloji ve klinik spektrum ve bu hastalıkların ayı-rıcı tanısı olgu senaryoları eşliğinde son literatür verileri ışığında detaylı olarak tartışıl-maktadır.
Keywords
histaminerjik anjiyoödem ürtiker herediter anjiyoödem C1inhibitor faktör XII anjiyopoietin-1 plazminojen
References
- Kaynaklar 1- Misra L, Khurmi N, Trentman TL. Angioedema: Classification,management and emerging therapies for the perioperativephysician. Indian J Anaesth. 2016; 60(8):534-41. 2- Kaplan AP, Greaves MW. Angioedema. J Am Acad Dermatol.2005; 53(3):373-88. 3- Wu MA, Perego F, Zanichelli A, Cicardi M. Angioedema phe-notypes: disease expression and classification. Clin Rev AllergyImmunol 2016; 51(2):162-9. 4- Pattanaik D, Lieberman JA. Pediatric angioedema. Curr Al-lergy Asthma Rep. 2017; 17(9):60. 5- Cicardi M, Suffritti C, Perego F, Caccia S. Novelties in the diag-nosis and treatment of angioedema. J Investig Allergol Clin Im-munol 2016; 26 (4): 212 -21. 6- Jaiganesh T, Wiese M, Hollingsworth J, Hughan C, Kamara M,Wood P, et al. Acute angioedema: recognition and management inthe emergency department. Eur J Emerg Med. 2013; 20(1):10-7. 7- Rye Rasmussen EH, Bindslev-Jensen C, Bygum A. Angioedema--assessment and treatment. Tidsskr Nor Laegeforen. 2012;132(21): 2391-5. 8- Gill P, Betschel SD. The clinical evaluation of angioedema. Immunol Allergy Clin North Am. 2017; 37(3):449-66. 9- LoVerde D, Files DC, Krishnaswamy G. Angioedema. Crit CareMed. 2017; 45(4):725-35. 10- Triggianese P, Guarino MD, Pellicano C, Borzi M, Greco E,Modica S, et al. Recurrent angioedema: Occurrence, features,and concomitant diseases in an İtalian single-center study. IntArch Allergy Immunol. 2017; 172(1):55-63. 11- Bouillet L, Boccon-Gibod I, Berard F, Nicolas JF. Recurrentangioedema: diagnosis strategy and biological aspects. Eur JDermatol 2014; 24(3):293-6. 12- Lewis LM. Angioedema: etiology, pathophysiology, currentand emerging therapies. J Emerg Med. 2013; 45(5):789-96. 13-James C, Bernstein JA. Current and future therapies for the tre-atment of histamine-induced angioedema. Expert Opin Phar-macother 2017; 18(3):253-62. 14- Bernstein JA, Lang DM, Khan DA, et al: The diagnosis andmanagement of acute and chronic urticaria: 2014 update. J Al-lergy Clin Immunol 2014;133:1270-7 15- Powell RJ, Leech SC, Till S,Huber PAJ, Nasser SM, ClarkAT.BSACI guideline for the management of chronic urticaria andangioedema. Clin Exp Allergy. 2015; 45:547–65. 16- Maurer M, Magerl M, Ansotegui I, Aygören-Pürsün E, Betsc-hel S, Bork K, et al. The international WAO/EAACI guidelinefor the management of hereditary angioedema-The 2017 revisi-on and update. Allergy 2018; 73(8):1575-96. 17- Huston DP, Sabato V. Decoding the enigma of urticaria andangioedema. J Allergy Clin Immunol Pract. 2018; 6(4):1171-5. 18- Giavina-Bianchi P, Aun MV, Motta AA, Kalil J, Castells M.Classification of angioedema by endotypes. Clin Exp Allergy2015; 45(6):1142-3. 19- Bas M, Adams V, Suvorava T, Niehues T, Hoffmann TK, KojdaG. Nonallergic angioedema: role of bradykinin. Allergy. 2007;62(8):842-56. 20- Busse PJ, Smith T. Histaminergic Angioedema. Immunol Al-lergy Clin North Am. 2017; 37(3):467-81. 21- Moellman JJ, Bernstein JA, Lindsell C, Banerji A, Busse PJ,Camargo CA Jr, et al. A consensus parameter for the evaluati-on and management of angioedema in the emergency depart-ment. Acad Emerg Med. 2014; 21(4):469-84. 22- Barbara DW, Ronan KP, Maddox DE, Warner MA. Periopera-tive angioedema: background, diagnosis, and management. JClin Anesth 2013; 25(4):335-43. 23-Zuberbier T, Bernstein JA. A Comparison of the United Statesand international perspective on chronic urticaria guidelines. JAllergy Clin Immunol Pract. 2018; 6(4):1144-51. 24- Cicardi M, Aberer W, Banerji A, Bas M, Bernstein JA, Bork K,et al. Classification, diagnosis, and approach to treatment forangioedema: consensus report from the Hereditary Angioede-ma International Working Group. Allergy 2014; 69(5):602-16 25- James C, Bernstein JA. Current and future therapies for thetreatment of histamine-induced angioedema. Expert OpinPharmacother 2017; 18(3):253-62. 26- Mansi M, Zanichelli A, Coerezza A, Suffritti C, Wu MA, Vacchi-ni R, et al. Presentation, diagnosis and treatment of angioede-ma without wheals: a retrospective analysis of a cohort of 1058patients. J Intern Med. 2015; 277(5):585-93. 27-Malbrán E, Fernández Romero D, Juri MC, Larrauri BJ,Malbrán A. Epidemiology of angioedema without wheals in anallergy and immunology center. Medicina (B Aires). 2015;75(5):273-6. 28-Faisant C, Boccon-Gibod I, Mansard C, Dumestre Perard C,Pralong P, Chatain C, et al. Idiopathic histaminergic angioede-ma without wheals: a case series of 31 patients. Clin Exp Im-munol. 2016; 185(1): 81-5. 29-Eli M, Joseph M, Kuznik B, Menachem S. Chronic idiopathicangioedema: a single center experience. Int J Dermatol 2014;53(10):e421-7. 30-Bucher MC, Petkovic T, Helbling A, Steiner UC. Idiopathic non-his-taminergic acquired angioedema: a case series and discussion ofpublished clinical trials. Clin Transl Allergy. 2017;7:27. 31-Hahn J, Hoffmann TK, Bock B, Nordmann-Kleiner M, Trainotti S,Greve J. Angioedema. Dtsch Arztebl Int. 2017; 114(29-30):489-96. 32- Bork K, Wulff K, Witzke G, Hardt J. Hereditary angioedemawith normal C1-INH with versus without specific F12 genemutations. Allergy 2015;70(8):1004-12. 33- Lumry WR. Overview of epidemiology, pathophysiology, anddisease progression in hereditary angioedema. Am J ManagCare 2013; 19(7 Suppl):s103-10. 34- Cugno M, Zanichelli A, Foieni F, Caccia S, Cicardi M. C1-in-hibitor deficiency and angioedema: molecular mechanismsand clinical progress. Trends Mol Med. 2009;15(2):69-78. 35- Zuraw BL, Christiansen SC. HAE pathophysiology and underl-ying mechanisms. Clin Rev Allergy Immunol. 2016; 51(2):216-29. 36- Caccia S, Suffritti C, Cicardi M. Pathophysiology of hereditary angi-oedema. Pediatr Allergy Immunol Pulmonol. 2014; 27(4):159-63. 37- Zuraw BL. Hereditary angioedema with normal C1 inhibitor: Fourtypes and counting. J Allergy Clin Immunol. 2018; 141(3):884-5. 38- Zeerleder S, Levi M. Hereditary and acquired C1-inhibitor-de-pendent angioedema: from pathophysiology to treatment. AnnMed. 2016; 48(4):256-67. 39- Kaplan AP. Enzymatic pathways in the pathogenesis of he-reditary angioedema: the role of C1 inhibitor therapy. J AllergyClin Immunol. 2010; 126(5):918-25. 40- Schmaier AH. The contact activation and kallikrein/kininsystems: pathophysiologic and physiologic activities. J ThrombHaemost. 2016; 14(1):28-39. 41- Charlesworth EN. Differential diagnosis of angioedema. Al-lergy Asthma Proc. 2002; 23(5):337-9. 42- Kaplan AP, Joseph K. The bradykinin-forming cascade and itsrole in hereditary angioedema. Ann Allergy Asthma Immunol.2010; 104(3):193-204. 43- Cicardi M, Zuraw BL. Angioedema due to bradykinin dysregu-lation. J Allergy Clin Immunol Pract. 2018; 6(4):1132-41. 44-Cicardi M, Zanichelli A. Diagnosing angioedema. Immunol Al-lergy Clin North Am. 2013; 33(4): 449-56. 45-Bohra S, Kariya PB, Bargale SD, Kiran S. Clinicopathologicalsignificance of Melkersson-Rosenthal syndrome. BMJ CaseRep. 2015 Jul 31;2015. pii: bcr2015210138. 46- Sher J, Davis-Lorton M. Angioedema with normal laboratory values:the next step. Curr Allergy Asthma Rep 2013; 13(5):563-70.
Details
| Primary Language | Turkish |
|---|---|
| Journal Section | makale |
| Authors | |
| Publication Date | March 7, 2019 |
| Published in Issue | Year 2019 Volume: 11 Issue: 2 |
