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Angioedema

Angioedema (BE: angiooedema), also known by its eponym Quincke's edema and the older term angioneurotic edema, is the rapid swelling (edema) of the skin, mucosa and submucosal tissues. Apart from the common form, mediated by allergy, it has been reported as a side effect of some medications, specifically ACE inhibitors. Additionally, there is an inherited form, due to deficiency of the blood protein C1-inhibitor. This form is called hereditary angioedema (HAE) or hereditary angio-neurotic edema (HANE), which is due to C1-esterase inhibitor deficiency. more...

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Medicines

Cases where angioedema progresses rapidly should be treated as a medical emergency as airway obstruction and suffocation can occur. Rapid treatment with epinephrine, often with an epi-pen, can be life-saving.

Signs and symptoms

The skin of the face, normally around the mouth, and the mucosa of the mouth and/or throat, as well as the tongue, swell up over the period of minutes to several hours. The swelling can also occur elsewhere, typically in the hands. Sometimes, there has been recent exposure to an allergen (e.g. peanuts), and urticaria (hives) develop simultaneously, but many times the cause is idiopathic (unknown). The swelling can be itchy. There may also be slightly decreased sensation in the affected areas due to compression of the nerves.

In severe cases, stridor of the airway occurs, with gasping inspiratory breath sounds and decreasing oxygen levels. Intubation and rapid treatment with epinephrine and antihistamines is required in these situations.

In hereditary angioedema, there is often no direct identifiable cause, although mild trauma and other stimuli can cause attacks. There is usually no associated itch or urticaria. Patients with this syndrome can also have attacks of recurrent abdominal pain, sometimes leading to an unnecessary laparotomy. There is also an increased incidence of autoimmune disease (e.g. lupus erythematosus, glomerulonephritis and hypothyroidism) due to altered activity of the complement system.

Diagnosis

The diagnosis is made on the clinical picture. When the patient has been stabilized, complement levels, especially C1-inhibitor and depletion of complement factors 2 and 4, may indicate the presence of hereditary angioedema (see below). Additionally, allergy testing should be undertaken to determine if any allergens need to be avoided in the future. If the patient was on ACE inhibitor medication, this has to be discontinued.

Pathophysiology

The final common pathway for the development of angioedema seems to be the activation of the bradykinin pathway. This peptide is a potent vasodilator, leading to rapid accumulation of fluid in the interstitium. This is most obvious in the face, where the skin has relatively little supporting connective tissue, and edema develops easily. Bradykinin is released by various cell types in response to numerous different stimuli; it is also a pain mediator.

Various mechanisms that interfere with bradykinin production or degradation can lead to angioedema. ACE inhibitors block the function of kininase II, the enzyme that degrades bradykinin. In hereditary angioedema, bradykinin formation is caused by continuous activation of the complement system due to a deficiency in on of its prime inhibitors, C1-esterase inhibitor (C1INH), and continuous production of kallikrein, another process inhibited by C1INH. This serine protease inhibitor (serpin) normally inhibits the conversion of C1 to C1r and C1s, which - in turn - activate other proteins of the complement system. Additionally, it inhibits various proteins of the coagulation cascade, although effects of its deficiency on the development of hemorrhage and thrombosis appear to be limited.

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C1 inhibitor infusion modifies platelet activity in hereditary angioedema patients
From Archives of Pathology & Laboratory Medicine, 7/1/02 by Coppola, Ludovic

* Context.-Cl inhibitor (Cl-INH) is an alpha^sub 2^-globulin that blocks esterolytic activity of the first component of the classic complement cascade. The alpha-granules of normal human platelets also contain CL-INH, which is expressed on the platelet surface during platelet secretion in healthy patients, but it is clearly reduced in patients with hereditary angioedema (HAE).

Objective.-To evaluate the effects of in vivo C1l-INH concentrate infusion on platelet responsiveness and coagulation system activity in patients with HAE.

Design.-Assessment of the platelet activity and plasma levels of Cl-INH, activated factor XII (XIla), and prothrombin fragment Fl.2 (Fl.2) before and after infusion of 15 U/ kg of Cl-INH concentrate.

Patients.tin 6 patients (4 men and 2 women), HAE was diagnosed according to the accepted clinical and laboratory criteria.

Measurements.-Platelet aggregation (final concentra

tions: adenosine diphosphate, 0.5, 1.25, and 2.5(mu)LM; collagen, 5 (mu)g//mL), CI-INH antigen (radial immunodiffusion), CI-INH activity (chromogenic substrates), and Xlla and Fl.2 (enzyme-linked immunosorbent assay).

Results.-After Cl-INH infusion, we observed a prompt increase of CI-INH level and a slow return toward its plasma preinfusion values within 4 to 7 days, a significant decrease of both adenosine diphosphate- and collagen-induced platelet aggregation versus preinfusion values (maximum after 1-2 days; P

Conclusions.-These data show a role of C1-INH in the control of platelet activity and that its deficiency increases platelet aggregability and plasma levels of Xlla and Fl.2 in patients with HAE.

(Arch Pathol Lab Med. 2002;126:842-845)

References

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10. Gadek JE, Hosea SW, Gelfand JA, et al. Replacement therapy in hereditary angioedema: successful treatment of acute episodes of angioedema with partly purified C1inhibitor, N Engl / Med. 1980;302:542-546,

11. Agostoni A. Inherited Cl inhibitor deficiency. Complement Inflamm. 1989; 6:112-118.

12. Waytes AT, Rosen FS, Frank MM. Treatment of hereditary angioedema with a vapor-heated C1inhibitor concentrate. N Engl] Med. 1996;334:1630-1634.

13. Fiane AE, Videm V, Johansen HT, Mellbye 01, Nielsen EW, Moliness TE. Cl-inhibitor attenuates hyperacute rejection and inhibits complement, leukocyte and platelet activation in an ex vivo pig-to-human perfusion model. Immunopharmacology. 1999;42:231-243.

14. Strong CJH, Cholin S, Spragg 1, et al. Angioedema induced by a peptide derived from the complement component C,. J Exp Med. 1988;168:1685.

15. Shapira M, Silver LD, Scott CF, et al. Prekallikrein activation and high molecular weight kininogen consumption in hereditary angioedema. N Engl Med. 1983;308:1050.

16. Coppola L, Tirelli A, Giunta R, Verrazzo G, D'Onofrio F A new approach to the hereditary angioedema. Adv Ther. 1990;7:229-234.

17. Coppola L, Tirelli A, Giunta R, Verrazzo G, D'Onofrio F. The usefulness of the antiplatelet drugs for prophylactic treatment in hereditary angioedema. in: Proceedings of the 61st European Atherosclerosis Society. Naples: Ariello Bros Press; 1993:167-168.

18. Wuillenin WA, Hack CE, Bleeker WK, Biemond B), Levi M, ten Cate H. Inactivation of factor XIa in vivo: studies in chimpanzees and in humans. Thromb Haemost. 1996;76:549-555.

19. Wuillenin WA, Minnema N, Meijers JCM, et al. Inactivation of factor Xia in human plasma assessed by measuring factor Xla-protease inhibitor complexes: major role for Cl -inhibitor. Blood. 1995;85:1517-1526.

20. Lammle B, Griffin JH. Formation of the fibrin clot: the balance of procoagulant and inhibitory factors. Clin Haematol. 1985;14:281-342.

21. Hack CE, Voerman HI, Eisele B, et al. Cl-esterase inhibitor substitution in sepsis. Lancet. 1992;339:378.

22. Coppola R, Cristilli P, Cugno M, Ariens RAS, Mari D, Mannucci PM. Measurement of activated factor XII in health and disease. Blood Coagul Fibrinolysis. 1996;7:530-535.

23. Dempfle CE, Pfitzener SA, Fahnle M, Heene DL. Measurement of factor Xlla and factor Vila allows early diagnosis of blood coagulation activation. Thromb Haemost. 1995;73:1434.

24. Mesters RM, Mannucci PM, Coppola R, Keller T, Ostermann H, Kienast 1. Factor VIla and antithrombin III activity during severe sepsis and septic shock in neutropenic patients. Blood. 1996;88:881-886.

25. Ford RP, Esnouf MP, Burgess Al, Sarphie A. An enzyme-linked immunosorbent assay (ELISA) for the measurement of activated factor XII (Hageman factor) in human plasma. I Immunoassay. 1996;17:119-131.

26. Pelzer H, Schwart A, Stuber W. Determination of human prothrombin activation fragment 1 +2 in plasma with an antibody against a synthetic peptide. Thromb Haemost. 1991;65:153-159.

27. Mannucci PM, Tripodi A, Bottasso B, et al. Markers of procoagulant in balance with inherited thrombophilic syndromes. Thromb Haemost. 1992;67: 200-207.

28. Donaldson VH, Mitchell BH, Everson B, Ratnoff OD. Interactions of Cl inhibitors from normal persons and patients with type II hereditary angioneurotic edema with purified activated Hageman factor (factor XIla). Blood. 1990;75:911921.

Ludovico Coppola, MD; Salvatore Guastafierro, MD; Giovanni Verrazzo, MD; Antonino Coppola, MD; Domenico De Lucia, MD; Angelo Trelli, MD

Accepted for publication February 18, 2002.

From the Department of Gerontology, Geriatric and Metabolic Diseases (Drs L. Coppola, Verrazzo, and A. Coppola), Department of Hematology, Transfusion Medicine, and Transplant Immunology (Drs Guastafierro and Tirelli), and Department of Laboratory Medicine (Dr De Lucia), Second University of Naples, Naples, Italy.

Reprints: Salvatore Guastafierro, MD, Department of Hematology, Second University of Naples, Largo Madonna delle Grazie, 2, 80138 Napoli, Italy (e-mail: ematologia@libero.it).

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