Parameters of the classical complement pathway predict disease severity in hereditary angioedema

Clinical Immunology

Volumn 139, Issue 1, 2011, Pages 85-93

  Csuka, Dorottya ^a   Füst, George ^a   Farkas, Henriette ^a   Varga, Lilian ^a  

^a SEMMELWEIS UNIVERSITY OF MEDICINE   (Hungary)

Author keywords

C1 inhibitor deficiency; C1rC1sC1 inh; C3bBbP; Disease marker; Hereditary angioedema; SC5b 9

Indexed keywords

ALTERNATIVE COMPLEMENT PATHWAY C3 C5 CONVERTASE; COMPLEMENT COMPONENT C1S; COMPLEMENT COMPONENT C1S INHIBITOR; DANAZOL; TRANEXAMIC ACID; ANTIESTROGEN; GENETIC MARKER;

ADOLESCENT; ADULT; AGED; AMPOULE; ANGIONEUROTIC EDEMA; ARTICLE; CHILD; COMPLEMENT CLASSICAL PATHWAY; CONTROLLED STUDY; DISEASE SEVERITY; FEMALE; HUMAN; MAJOR CLINICAL STUDY; MALE; PREDICTIVE VALUE; PRESCHOOL CHILD; PRIORITY JOURNAL; PROPHYLAXIS; SCHOOL CHILD; ANGIOEDEMAS, HEREDITARY; GENETIC MARKER; GENETICS; METABOLISM; MIDDLE AGED; PHYSIOLOGY; YOUNG ADULT;

ADOLESCENT; ADULT; AGED; ANGIOEDEMAS, HEREDITARY; CHILD; CHILD, PRESCHOOL; COMPLEMENT PATHWAY, CLASSICAL; DANAZOL; ESTROGEN ANTAGONISTS; FEMALE; GENETIC MARKERS; HUMANS; MALE; MIDDLE AGED; YOUNG ADULT;

EID: 79952997775     PISSN: 15216616     EISSN: 15217035     Source Type: Journal    
DOI: 10.1016/j.clim.2011.01.003     Document Type: Article

References (37)

1. Agostoni A., Aygoren-Pursun E., Binkley K.E., et al. Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. J. Allergy Clin. Immunol. 2004, 114(3 Suppl):S51-S131.
2. Davis A.E. The pathophysiology of hereditary angioedema. Clin. Immunol. 2005, 114(1):3-9.
3. Frank M.M. Hereditary angioedema. J. Allergy Clin. Immunol. 2008, 121(2 Suppl):S398-S401. (quiz S19).
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):267-274.
5. Frank M.M., Gelfand J.A., Atkinson J.P. Hereditary angioedema: the clinical syndrome and its management. Ann. Intern. Med. 1976, 84(5):580-593.
6. Agostoni A. Inherited C1 inhibitor deficiency. Complement Inflamm. 1989, 6(2):112-118.
7. Lung C.C., Chan E.K., Zuraw B.L. Analysis of an exon 1 polymorphism of the B2 bradykinin receptor gene and its transcript in normal subjects and patients with C1 inhibitor deficiency. J. Allergy Clin. Immunol. 1997, 99(1 Pt 1):134-146.
8. Duponchel C., Djenouhat K., Fremeaux-Bacchi V., Monnier N., Drouet C., Tosi M. Functional analysis of splicing mutations and of an exon 2 polymorphic variant of SERPING1/C1NH. Hum. Mutat. 2006, 27(3):295-296.
9. Blasko B., Szeplaki G., Varga L., et al. Relationship between copy number of genes (C4A, C4B) encoding the fourth component of complement and the clinical course of hereditary angioedema (HAE). Mol. Immunol. 2007, 44(10):2667-2674.
10. Drouet C., Desormeaux A., Robillard J., et al. Metallopeptidase activities in hereditary angioedema: effect of androgen prophylaxis on plasma aminopeptidase P. J. Allergy Clin. Immunol. 2008, 121(2):429-433.
11. Farkas H., Fust G., Fekete B., Karadi I., Varga L. Eradication of Helicobacter pylori and improvement of hereditary angioneurotic oedema. Lancet 2001, 358(9294):1695-1696.
12. Visy B., Fust G., Varga L., et al. Sex hormones in hereditary angioneurotic oedema. Clin. Endocrinol. (Oxf) 2004, 60(4):508-515.
13. Bork K., Bygum A., Hardt J. Benefits and risks of danazol in hereditary angioedema: a long-term survey of 118 patients. Ann. Allergy Asthma Immunol. 2008, 100(2):153-161.
14. Bork K., Hardt J. Hereditary angioedema: increased number of attacks after frequent treatments with C1 inhibitor concentrate. Am. J. Med. 2009, 122(8):780-783.
15. Z. Kelemen, D. Moldovan, E. Mihaly, et al., Baseline level of functional C1-inhibitor correlates with disease severity scores in hereditary angioedema. Clin. Immunol. 134(3) (2010) 354-358.
16. K. Joseph, T.E. Tholanikunnel, A.P. Kaplan, Treatment of episodes of hereditary angioedema with C1 inhibitor: serial assessment of observed abnormalities of the plasma bradykinin-forming pathway and fibrinolysis. Ann. Allergy Asthma Immunol. 104(1) (2010) 50-54.
17. Davis A.E. C1 inhibitor and hereditary angioneurotic edema. Annu. Rev. Immunol. 1988, 6:595-628.
18. Ghebrehiwet B., Randazzo B.P., Dunn J.T., Silverberg M., Kaplan A.P. Mechanisms of activation of the classical pathway of complement by Hageman factor fragment. J. Clin. Invest. 1983, 71(5):1450-1456.
19. Cugno M., Nuijens J., Hack E., Eerenberg A., Frangi D., Agostoni A., Cicardi M. Plasma levels of C1- inhibitor complexes and cleaved C1- inhibitor in patients with hereditary angioneurotic edema. J. Clin. Invest. 1990, 85(4):1215-1220.
20. Cugno M., Hack C.E., de Boer J.P., Eerenberg A.J., Agostoni A., Cicardi M. Generation of plasmin during acute attacks of hereditary angioedema. J. Lab. Clin. Med. 1993, 121(1):38-43.
21. Nielsen E.W., Johansen H.T., Gaudesen O., Osterud B., Olsen J.O., Hogasen K., Hack C.E., Mollnes T.E. C3 is activated in hereditary angioedema, and C1/C1-inhibitor complexes rise during physical stress in untreated patients. Scand. J. Immunol. 1995, 42(6):679-685.
22. Cugno M., Cicardi M., Bottasso B., Coppola R., Paonessa R., Mannucci P.M., Agostoni A. Activation of the coagulation cascade in C1-inhibitor deficiencies. Blood 1997, 89(9):3213-3218.
23. Cugno M., Zanichelli A., Bellatorre A.G., Griffini S., Cicardi M. Plasma biomarkers of acute attacks in patients with angioedema due to C1-inhibitor deficiency. Allergy 2009, 64(2):254-257.
24. Cedzynski M., Madalinski K., Gregorek H., et al. Possible disease-modifying factors: the mannan-binding lectin pathway and infections in hereditary angioedema of children and adults. Arch. Immunol. Ther. Exp. (Warsz) 2008, 56(1):69-75.
25. Nussberger J., Cugno M., Amstutz C., Cicardi M., Pellacani A., Agostoni A. Plasma bradykinin in angio-oedema. Lancet 1998, 351(9117):1693-1697.
26. Ziccardi R.J. The first component of human complement (C1): activation and control. Springer Semin. Immunopathol. 1983, 6(2-3):213-230.
27. Cooper N.R. The classical complement pathway: activation and regulation of the first complement component. Adv. Immunol. 1985, 37:151-216.
28. Laurell A.B., Martensson U., Sjoholm A.G. C1 dissociation. Spontaneous generation in human serum of a trimer complex containing C1 inactivator, activated C1r, and zymogen C1s. J. Immunol. 1987, 139(12):4145-4151.
29. Ghebrehiwet B., Randazzo B.P., Kaplan A.P. Studies of complement autoactivatability in hereditary angioedema: direct relationship to functional C-1-INA and the effect of classical pathway activators. Clin. Immunol. Immunopathol. 1984, 32(1):101-110.
30. Windfuhr J.P., Alsenz J., Loos M. The critical concentration of C1-esterase inhibitor (C1-INH) in human serum preventing auto-activation of the first component of complement (C1). Mol. Immunol. 2005, 42(6):657-663.
31. Bowen T., Cicardi M., Farkas H., et al. 2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema. Allergy Asthma Clin. Immunol. 2010, 6(1):24.
32. Kalmar L., Bors A., Farkas H., Vas S., Fandl B., Varga L., Fust G., Tordai A. Mutation screening of the C1 inhibitor gene among Hungarian patients with hereditary angioedema. Hum. Mutat. 2003, 22(6):498.
33. Bowen T., Cicardi M., Farkas H., et al. Canadian 2003 international consensus algorithm for the diagnosis, therapy, and management of hereditary angioedema. J. Allergy Clin. Immunol. 2004, 114(3):629-637.
34. Kerenyi A., Nagy G., Veres A., et al. C1r-C1s-C1inhibitor (C1rs-C1inh) complex measurements in tears of patients before and after penetrating keratoplasty. Curr. Eye Res. 2002, 24(2):99-104.
35. Deppisch R., Schmitt V., Bommer J., Hansch G.M., Ritz E., Rauterberg E.W. Fluid phase generation of terminal complement complex as a novel index of bioincompatibility. Kidney Int. 1990, 37(2):696-706.
36. Cicardi M., Bergamaschini L., Cugno M., Hack E., Agostoni G., Agostoni A. Long-term treatment of hereditary angioedema with attenuated androgens: a survey of a 13-year experience. J. Allergy Clin. Immunol. 1991, 87(4):768-773.
37. Fure H., Nielsen E.W., Hack C.E., Mollnes T.E. A neoepitope-based enzyme immunoassay for quantification of C1-inhibitor in complex with C1r and C1s. Scand. J. Immunol. 1997, 46(6):553-557.