Gökhan AYTEKİN, Fatih ÇÖLKESEN, Eray YILDIZ, Şevket ARSLAN

A patient with hereditary angioedema and systemic lupus erythematosus: Coincidence or coexistence?

Angioedema is classified into two major groups: mast cell-mediated (histaminergic) and bradykinin-mediated angioedema. Hereditary angioedema and acquired angioedema are well-defined groups of bradykinin-mediated angioedema. Both hereditary angioedema and acquired angioedema demonstrate a similar clinical presentation and can be challenging to differentiate. The prevalence of autoimmune diseases is higher in patients with HAE and lupus is an autoimmune disease associated with HAE. In this case, we aimed to present a male patient who was monitored with the diagnosis of HAE and developed systemic lupus erythematosus (SLE) in the follow-up and to discuss whether the patient had hereditary angioedema and concomitant lupus, or acquired angioedema secondary to SLE.

PDF

___

1. Lipsker D, Hauptmann G. Cutaneous manifestations of complement deficiencies. Lupus. 2010;19:1096-106.

2. Nzeako UC, Frigas E, Tremaine WJ. Hereditary angioedema: a broad review for clinicians. Arch Int Med. 2001;161:2417-29.

3. Brickman CM, Tsokos GC, Balow JE, et al. Immunoregulatory disorders associated with hereditary angioedema. I. Clinical manifestations of autoimmune disease. J Allergy Clin Immunol. 1986;77:749-57.

4. Khan S, Tarzi MD, Dore PC, et al. Secondary systemic lupus erythematosus: an analysis of 4 cases of uncontrolled hereditary angioedema. Clin immunology. 2007;123:14-7.

5. Kohler PF, Percy J, Campion WM, et al. Hereditary angioedema and “familial” lupus erythematosus in identical twin boys. Am J Med. 1974;56:406-11.

6. Cicardi M, Aberer W, Banerji A, et al. Classification, diagnosis, and approach to treatment for angioedema: consensus report from the Hereditary Angioedema Int Working Group. Allerg. 2014;69:602-16.

7. Fremeaux-Bacchi V, Guinnepain MT, Cacoub P, et al. Prevalence of monoclonal gammopathy in patients presenting with acquired angioedema type 2. Am J Med. 2002;113:194-9.

8. Bork K, Meng G, Staubach P, Hardt J. Hereditary angioedema: new findings concerning symptoms, affected organs, and course. Am J Med. 2006;119:267- 74.

9. Zanichelli A, Azin GM, Wu MA, Suffritti C, Maggioni L, Caccia S, et al. Diagnosis, Course, and Management of Angioedema in Patients With Acquired C1-Inhibitor Deficiency. J Allerg Clin Immunol Practice. 2017;5:1307-13.

10. Donaldson VH, Hess EV, McAdams AJ. Lupus-erythematosus-like disease in three unrelated women with hereditary angioneurotic edema. Ann Int Med. 1977;86:312-3.

11. Gallais Serezal I, Bouillet L, Dhote R, et al. Hereditary angioedema and lupus: A French retrospective study and literature review. Autoimmunity Reviews. 2015;14:564-8.

12. Massa MC, Connolly SM. An association between C1 esterase inhibitor deficiency and lupus erythematosus: report of two cases and review of the literature. J Am Academy Dermatol. 1982;7:255-64.

13. Pickering MC, Botto M, Taylor PR, etal. Systemic lupus erythematosus, complement deficiency, and apoptosis. Advances Immunol. 2000;76:227-324.

14. O’Neil KM. Complement deficiency. Clin Reviews Allerg & Immunol. 2000;19:83-108.

15. Suzuki Y, Nihei H, Mimura N, et al. A case of hereditary angioneurotic edema associated with systemic lupus erythematosus. Japanese J Med. 1986;25:281- 7.

16. Carpenter CB, Ruddy S, Shehadeh IH, et al. Complement metabolism in man: hypercatabolism of the fourth (C4) and third (C3) components in patients with renal allograft rejection and hereditary, angioedema (HAE). J Clin Investigation. 1969;48:1495-505.