Therapy of hereditary angioedema: is it time to focus on a different strategic approach? psychosocial issues and stress management
Keywords:Bradykinin, Chronic disorder, CI Inhibitor, Economic burden, New medications, Stress, Quality of life
Hereditary angioedema is an autosomal dominant genetic disorder due to the deficiency of CI esterase inhibitor with elevated levels of bradykinin. The disorder was named hereditary angioneurotic edema because of the associated mental stress which exacerbated the condition. It manifests most often as edema of the skin and mucosal surfaces. Laryngeal edema can cause fatal obstruction. The various treatment modalities available for HAE includes plasma derived C1-INH concentrate, ecallantide, icatibant, attenuated androgens and fresh frozen plasma. The drugs for HAE are efficacious, but with side effects and are expensive. Patients with HAE exhibit anxiety and depression, and the stressful state by itself predisposes to further attacks which in the long run forms a vicious cycle one leading on to the other. Considering the fact that HAE is a chronic disorder needing meticulous attention, together with the task of home therapy, availability of medication, adverse effects of drugs, the economic burden and the undue compromise on quality of life, there definitely arises a need for focus on a different strategic approach towards treatment. Currently the focus is on various treatment modalities targeting the complement system and the acute symptomatology of the condition, rather than on the “neurotic component or the stress factor” which might prove to be one major area of therapeutic benefit. Management of stress related factors, overcoming anxiety and depression through psychosocial modalities and simple alternative therapies like yoga as a part of routine activity can greatly reduce the disease, as well as the economic burden and improve the quality of life.
Joshua A. Boyce K. Frank Austen. Disorders of Immune Mediated Injury: Allergies, Anaphylaxis and Systemic Mastocytosis. In: DL Kasper, AS Fauci, SL Hauser, DL Longo, JL Jameson, JL Loscalzo, eds. Harrison’s Principles of Internal Medicine. 19th ed. New York, NY: Mcgraw Hill; 2015:2113-2124.
Kumar V, Abbas AK, Jon C. Aster. Robbins and Cotran Pathologic Basis of Disease. 8th ed. Philadelphia, PA: Elsevier/Saunders; 2007:183-257.
Bowen T, Cicardi M, Farkas H. International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema. Allergy Asthma Clin-Immunol. 2010;6(1):24.
Douglas T. Johnston, DO. Diagnosis and Management of Hereditary Angioedema. J Am Osteopath Assoc. 2011;111(1):28-36.
Hemperly SE, Agarwal NS, Ying-Yang X, Yu-Xiang Z, Timothy J. Craig. Recent Advances in the Management of Hereditary Angioedema J Am Osteopath Assoc. 2013;113(7):546-55 .
Bouillet L, Longhurst H, Boccon-Gibod I. Disease expression in women with hereditary angioedema. Am J Obstet Gynecol. 2008;199(5):484.1-4.
Bork K, Meng G, Staubach P, Hardt J. Hereditary angioedema: new findings concerning symptoms, affected organs, and course. Am J Med. 2006;119(3):267-74.
Hannah D, Rafael F. Management of acute attacks of hereditary angioedema: role of ecallantide. J blood Medicine. 2015;6:115-23.
Zuraw BL, Busse PJ, Martha W, Joshua J, William L, Baker J, et al. Nanofiltered C1 Inhibitor Concentrate for Treatment of Hereditary Angioedema. N Engl J Med. 2010;363:513-22.
Neki NS, Tamil M. Hereditary angioedema. J Indian Acad Clin Med. 2014;15(3-4):210-15.
Gompels MM, Lock RJ, Gidofalvy E, Fust G, Varga L. A multicentre evaluation of the diagnostic efficiency of serological investigations for C1 inhibitor deficiency. J Clin Pathol. 2002;55:145-7.
Craig T, Pürsün EA, 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):18.
Longhurst HJ, Farkas H, Craig T. HAE international home therapy consensus document. Allergy, Asthma, and Clinical Immunology: Official Journal of the Canadian Society of Allergy and Clinical Immunology. 2010;6(1):22.
Martinez SI, Rusicke E, Aygoren PE, 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;203:131.1-7.
Caballero T, Farkas H, Bouillet L, Bowen T, Gompel A, 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 Immuno. 2012;129:308-20.
Lunn M, Santos C, Craig T. Cinryze TM. As the first approved C1 inhibitor in the USA for the treatment of hereditary angioedema: approval, efficacy and safety. J blood medicine. 2010;1:163-70.
FDA approves Berinert. Available at. https://www.drugs.com/newdrugs/csl-behring-announces-fda-approval-berinert-first-only-therapy-approved-acute-abdominal-facial-1681.html. Assessed 7 October 2017
CSL Behring receives FDA approval of expanded label on Beriert for self-administration and treatment of acute laryngeal attacks of hereditary angioedema. Available at: https://www.drugs.com/newdrugs/csl-behring-receives-fda-approval-expanded-label-berinert-self-administration-acute-laryngeal-3012. html. Assessed 7 October 2017
Zuraw BL, Banerji A, Bernstein JA. US hereditary angioedema association medical advisory board 2013 recommendations for the management of hereditary angioedema due to C1 inhibitor deficiency. J. Allergy Clin Immunol Prac. 2013;1:458-67.
Bernstein JA, Qazi M. Ecallantide: Its pharmacology, pharmacokinetics, clinical efficacy and tolerability. Expert Rev Clin Immunol. 2010;6(1):29-39.
Cicardi M, Banerji A, Bracho F. Icatibant, a new bradykinin- receptor antagonist, in hereditary angioedema. N Engl J Med. 2010;363(6):532-41.
Marietta M, Franchini M, Bindi ML, Picardi F, Ruggeri M, De Silvestro G. Is solvent/detergent plasma better than standard fresh-frozen plasma? A systematic review and an expert consensus document. Blood Transfusion. 2016;14(4):277-86.
Nzeako UC, Frigas E, Tremaine WJ. Hereditary Angioedema. A broad review for clinicians. Arch Intern Med. 2001;161: 2417-29.
Bork K, Pitton M, Harten P, Koch P. Hepatocellular adenomas in patients taking danazol for hereditary angiooedema. Lancet. 1999;353:1066-7.
Farkas H. Pediatric hereditary angioedema due to C1-inhibitor deficiency. Allergy, Asthma & Clinical Immunology. 2010;6(1):18.
Gompels MM, Lock RJ, Abinun M. C1 inhibitor deficiency: consensus document. Clin Exp Immunol. 2005;139:379-94.
New FDA approved drugs for 2017. Available at: https://www.centerwatch.com/drug-information/fda-approved-drugs/drug/100210/-haegarda-c1-esterase-inhibitor subcutaneous - human-. Assessed 7 October 2017
Caballero T, Canabal J, Rivero-Paparoni D, Cabañas R. Management of hereditary angioedema in pregnant women: a review. International Journal of Women’s Health. 2014;6:839-48.
Banerji A, Riedl M. Managing the female patient with hereditary angioedema. Womens Health. 2016;12(3):351-61.
Segerstrom SC, Miller GE. Psychological Stress and the Human Immune System: A Meta-Analytic Study of 30 Years of Inquiry. Psychol Bull. 2004;30(4):601-30.
Arora S, Bhattacharjee J. Modulation of immune responses in stress by Yoga. Int J of Yoga. 2008;1(2):45-55.