Acquired Angioedema Due to C1 Inhibitor Deficiency Questions & Answers

1. Caldwell JR, Ruddy S, Schur PH, Austen KF. Acquired C1 inhibitor deficiency in lymphosarcoma. Clin Immunol Immunopathol. 1972. 1:39-52.

2. Caballero T, Baeza ML, Cabañas R, 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(5):333-47; quiz follow 347. [QxMD MEDLINE Link].

3. Cugno M, Zanichelli A, Foieni F, Caccia S, Cicardi M. C1-inhibitor deficiency and angioedema: molecular mechanisms and clinical progress. Trends Mol Med. 2009 Feb. 15(2):69-78. [QxMD MEDLINE Link].

4. López-Lera A, Garrido S, Nozal P, Skatum L, Bygum A, Caballero T, et al. Serum complexes between C1INH and C1INH autoantibodies for the diagnosis of acquired angioedema. Clin Exp Immunol. 2019 Dec. 198 (3):341-350. [QxMD MEDLINE Link].

5. Bidkar VG, Rajan NR, Dasar S, Naik AS, Rao R. Acquired Angioedema: A Rare Manifestation of Angioimmunoblastic T Cell Lymphoma. Indian J Otolaryngol Head Neck Surg. 2019 Oct. 71 (Suppl 1):96-99. [QxMD MEDLINE Link].

6. Cugno M, Castelli R, Cicardi M. Angioedema due to acquired C1-inhibitor deficiency: a bridging condition between autoimmunity and lymphoproliferation. Autoimmun Rev. 2008 Dec. 8(2):156-9. [QxMD MEDLINE Link].

7. Castelli R, Wu MA, Arquati M, Zanichelli A, Suffritti C, Rossi D, et al. High prevalence of splenic marginal zone lymphoma among patients with acquired C1 inhibtor deficiency. Br J Haematol. 2016 Jan 5. [QxMD MEDLINE Link].

8. Roy S, Konala VM, Kyaw T, Chakraborty S, Naramala S, Gayam V, et al. An Unusual Case of Acquired Angioedema and Monoclonal Gammopathy of Renal Significance in a Middle-Aged Caucasian Female. J Investig Med High Impact Case Rep. 2020 Jan-Dec. 8:2324709620912096. [QxMD MEDLINE Link].

9. Banerji A, Sheffer AL. The spectrum of chronic angioedema. Allergy Asthma Proc. 2009 Jan-Feb. 30(1):11-6. [QxMD MEDLINE Link].

10. Cicardi M, Zanichelli A. Acquired angioedema. Allergy Asthma Clin Immunol. 2010 Jul 28. 6(1):14. [QxMD MEDLINE Link]. [Full Text].

11. Bouillet-Claveyrolas L, Ponard D, Drouet C, Massot C. Clinical and biological distinctions between type I and type II acquired angioedema. Am J Med. 2003 Oct 1. 115(5):420-1. [QxMD MEDLINE Link].

12. Engel R, Rensink I, Roem D, Brouwer M, Kalei A, Perry D, et al. ELISA to measure neutralizing capacity of anti-C1-inhibitor antibodies in plasma of angioedema patients. J Immunol Methods. 2015 Aug 28. [QxMD MEDLINE Link].

13. Bork K. An evidence based therapeutic approach to hereditary and acquired angioedema. Curr Opin Allergy Clin Immunol. 2014 Aug. 14 (4):354-62. [QxMD MEDLINE Link].

14. Zegers IH, Aaldering KN, Nieuwhof CM, Schouten HC. Non-myeloablative allogeneic stem cell transplantation: a new treatment option for acquired angioedema?. Neth J Med. 2015 Oct. 73 (8):383-5. [QxMD MEDLINE Link].

15. Bork K, Staubach-Renz P, Hardt J. Angioedema due to acquired C1-inhibitor deficiency: spectrum and treatment with C1-inhibitor concentrate. Orphanet J Rare Dis. 2019 Mar 13. 14 (1):65. [QxMD MEDLINE Link].

16. Gobert D, Paule R, Ponard D, Levy P, Frémeaux-Bacchi V, Bouillet L, et al. A nationwide study of acquired C1-inhibitor deficiency in France: Characteristics and treatment responses in 92 patients. Medicine (Baltimore). 2016 Aug. 95 (33):e4363. [QxMD MEDLINE Link].

17. Longhurst H, Cicardi M, Craig T, Bork K, Grattan C, Baker J, et al. Prevention of Hereditary Angioedema Attacks with a Subcutaneous C1 Inhibitor. N Engl J Med. 2017 Mar 23. 376 (12):1131-1140. [QxMD MEDLINE Link].

18. Levi M, Hack CE, van Oers MH. Rituximab-induced elimination of acquired angioedema due to C1-inhibitor deficiency. Am J Med. 2006 Aug. 119(8):e3-5. [QxMD MEDLINE Link].

19. Ziakas PD, Giannouli S, Psimenou E, Evangelia K, Tzioufas AG, Voulgarelis M. Acquired angioedema: a new target for rituximab?. Haematologica. 2004 Aug. 89(8):ELT13. [QxMD MEDLINE Link].

20. Klossowski N, Braun SA, von Gruben V, Losem C, Plewe D, Homey B, et al. [Acquired angioedema with C1-INH deficiency and accompanying chronic spontaneous urticaria in a patient with chronic lymphatic B cell leukemia]. Hautarzt. 2015 Sep 3. [QxMD MEDLINE Link].

21. Desai HG, Shah SS. Recurrent intestinal obstruction with acquired angio-oedema, due to C1-esterase inhibitor deficiency. J Assoc Physicians India. 2014 Jun. 62 (6):524-5. [QxMD MEDLINE Link].

22. Kaur R, Williams AA, Swift CB, Caldwell JW. Rituximab therapy in a patient with low grade B-cell lymphoproliferative disease and concomitant acquired angioedema. J Asthma Allergy. 2014. 7:165-7. [QxMD MEDLINE Link].

23. Dreyfus DH, Na CR, Randolph CC, Kearney D, Price C, Podell D. Successful rituximab B lymphocyte depletion therapy for angioedema due to acquired C1 inhibitor protein deficiency: association with reduced C1 inhibitor protein autoantibody titers. Isr Med Assoc J. 2014 May. 16 (5):315-6. [QxMD MEDLINE Link].

24. Levi M, Cohn D, Zeerleder S, Dziadzio M, Longhurst H. Long-term effects upon rituximab treatment of acquired angioedema due to C1-inhibitor deficiency. Allergy. 2019 Apr. 74 (4):834-840. [QxMD MEDLINE Link].

25. Rottem M, Mader R. Successful use of etanercept in acquired angioedema in a patient with psoriatic arthritis. J Rheumatol. 2010 Jan. 37(1):209. [QxMD MEDLINE Link].

Amanda T Moon, MD Clinical Assistant Professor of Dermatology, Children's Hospital of Philadelphia

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Melody Esmaeili MD Candidate, Perelman School of Medicine at the University of Pennsylvania

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Ru'aa Al Harithy, MBBS, FRCPC Clinical Fellow in Laser and Cosmetic Dermatology, Division of Dermatology, SunnyBrook Hospital, University of Toronto Faculty of Medicine, Canada

Ru'aa Al Harithy, MBBS, FRCPC is a member of the following medical societies: American Academy of Dermatology, Canadian Dermatology Association

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Warren R Heymann, MD Head, Division of Dermatology, Professor, Department of Internal Medicine, Rutgers New Jersey Medical School

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William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

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Robert A Schwartz, MD, MPH Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

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Michael J Wells, MD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

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