Complement-Related Disorders Medication

Updated: Jan 15, 2016
  • Author: M Michael Glovsky, MD; Chief Editor: Michael A Kaliner, MD  more...
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Medication

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and to prevent complications.

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Androgens, synthetic

Class Summary

In hereditary angioedema, stanozolol and danazol increase level of deficient C1-INH and prevent attacks. Danazol not recommended in children.

Danazol

Increases C4 levels and reduces attacks associated with angioedema.

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Kallikrein Inhibitor

Class Summary

Elicits specific kallikrein inhibitor activity resulting in bradykinin reduction. Useful for treating acute episodic attacks.

Ecallantide (Kalbitor)

Potent, selective, reversible inhibitor of plasma kallikrein. Binds to plasma kallikrein and blocks its binding site, inhibiting the conversion of high molecular weight kininogen to bradykinin. By directly inhibiting plasma kallikrein, reduces conversion of high molecular weight kininogen to bradykinin, thereby treating the disease during acute episodic attacks of hereditary angioedema (HAE). Indicated for treating acute HAE attacks.

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Blood Product Derivative

Class Summary

These agents are used for prophylaxis and treatment against attacks in adolescents and adults with hereditary or acquired angioedema.

C1 inhibitor, human (Cinryze, Berinert)

Serine proteinase inhibitor found in human blood that regulates activation of the complement pathway, intrinsic coagulation system, and fibrinolytic system. Binds to and neutralizes substrates that activate these systems, thereby suppressing activity. Available as a pasteurized, lyophilized preparation derived from purified human plasma. One unit corresponds to the mean quantity of C1 inhibitor present in 1 mL of normal fresh plasma. Indicated for acute abdominal and facial angioedema attacks in adolescents and adults with hereditary angioedema (HAE).

Cinryze is indicated for routine prophylaxis against attacks in adolescents and adults with hereditary or acquired angioedema.

Berinert is indicated for abdominal or facial HAE attacks.

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