Pathogenetic and clinical aspects of C1 inhibitor deficiency

Marco Cicardi, Luigi Bergamaschini, Massimo Cugno, Andrea Beretta, Lorenza C. Zingale, Monica Colombo, Angelo Agostoni

Research output: Contribution to journalArticle

82 Citations (Scopus)

Abstract

People deficient in C1-INH present recurrent angioedema localized to subcutaneous or mucous tissues. The defect can be caused by impaired synthesis, due to a genetic defect (hereditary angioedema), or by increased catabolism (acquired angioedema). In our experience the majority of patients with acquired angioedema (16 of 18) have autoantibodies to C1-INH in their serum. These autoantibodies bind to C1-INH with different and generally low affinity. The vasopermeability mediator responsible for attacks is still undefined: bradykinin (derived from cleavage of high molecular weight kininogen) and a kinin-like peptide (derived from the second component of complement) still remain the two primary candidates. We examined the systems controlled by C1-INH (complement, contact system, fibrinolysis and coagulation) and found that all of them are activated during angioedema attacks. Activation of the coagulation leads to generation of thrombin whose vasoactive effect can thus influence edema formation. Treatment of severe angioedema attacks is satisfactorily performed with C1-INH plasma concentrate although patients with an acquired defect frequently need very high doses. Attenuated androgens effectively prevent attacks in hereditary angioedema, but their safety, on the very long-term, needs to be further assessed. Acquired angioedema generally fail to respond to these drugs, but can be treated prophylactically with antifibrinolytic agents.

Original languageEnglish
Pages (from-to)366-376
Number of pages11
JournalImmunobiology
Volume199
Issue number2
Publication statusPublished - 1998

Fingerprint

Hereditary Angioedemas
Angioedema
Autoantibodies
High Molecular Weight Kininogens
Complement C1
Antifibrinolytic Agents
Kinins
Bradykinin
Fibrinolysis
Thrombin
Androgens
Edema
Safety
Peptides
Serum
Pharmaceutical Preparations
Acquired angioedema
Therapeutics

ASJC Scopus subject areas

  • Immunology

Cite this

Cicardi, M., Bergamaschini, L., Cugno, M., Beretta, A., Zingale, L. C., Colombo, M., & Agostoni, A. (1998). Pathogenetic and clinical aspects of C1 inhibitor deficiency. Immunobiology, 199(2), 366-376.

Pathogenetic and clinical aspects of C1 inhibitor deficiency. / Cicardi, Marco; Bergamaschini, Luigi; Cugno, Massimo; Beretta, Andrea; Zingale, Lorenza C.; Colombo, Monica; Agostoni, Angelo.

In: Immunobiology, Vol. 199, No. 2, 1998, p. 366-376.

Research output: Contribution to journalArticle

Cicardi, M, Bergamaschini, L, Cugno, M, Beretta, A, Zingale, LC, Colombo, M & Agostoni, A 1998, 'Pathogenetic and clinical aspects of C1 inhibitor deficiency', Immunobiology, vol. 199, no. 2, pp. 366-376.
Cicardi M, Bergamaschini L, Cugno M, Beretta A, Zingale LC, Colombo M et al. Pathogenetic and clinical aspects of C1 inhibitor deficiency. Immunobiology. 1998;199(2):366-376.
Cicardi, Marco ; Bergamaschini, Luigi ; Cugno, Massimo ; Beretta, Andrea ; Zingale, Lorenza C. ; Colombo, Monica ; Agostoni, Angelo. / Pathogenetic and clinical aspects of C1 inhibitor deficiency. In: Immunobiology. 1998 ; Vol. 199, No. 2. pp. 366-376.
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