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Managing C1 Esterase Inhibitor Deficiency: Effective Treatment Options

Learn about symptoms, management, and use of C1 esterase inhibitor in hereditary and acquired angioedema. Find dosing guidelines and side effects.

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Managing C1 Esterase Inhibitor Deficiency: Effective Treatment Options

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  1. C1 Esterase Inhibitor Deficiency Dr. C. Kalicinsky Allergy and Clinical Immunology

  2. Objectives • List the symptoms of C1 esterase inhibitor deficiency. • Contrast management of C1 esterase inhibitor deficiency with other forms of angioedema. • Become familiar with the use of C1 esterase inhibitor concentrate.

  3. C1 esterase inhibitor • Protein, which in its active form prevents fluid from leaving blood vessels, thereby preventing tissue swelling. This type of swelling is called angioedema.

  4. C1 Esterase Inhibitor Deficiency • Hereditary (HAE) • Acquired (AAE) - malignancy, especially hematologic - connective tissue disease • Diagnosed by measuring c1 esterase inhibitor level (Type 1) and function (Type 2) • Occasionally, the level and function are normal (Type 3) • Angioedema without urticaria • Can be fatal due to asphyxiation • Gut involvement causes severe abdominal pain

  5. Management • Epinephrine, antihistamine and corticosteroid NOT effective • Airway management • Once diagnosed, patients are given letter or wallet card explaining their condition and treatment to present to ER.

  6. C1 Esterase Inhibitor Concentrate (Berinert P) • Blood product, therefore blood product request form to be faxed to blood bank. • Some WRHA and rural hospital blood banks have a list of patients requiring this product and may keep a supply available. • WRHA monograph in final stages of approval and will be posted on Blood Manitoba website by Feb 2016. • Allergy on call needs to authorize release from local bloodbank.

  7. DOSAGE: Usual: 20 units /kg IV by slow IV injection (this is stated in product insert) Maximum single dose:1500 units (the maximum single dose may be increased beyond 1500 units if authorized by an immunologist)

  8. May repeat dose of 500-1000 units if condition does not improve in 1-2 hours. • Side effects: headache, abdominal pain, nausea, muscle spasm, pain, diarrhea, vomiting. Anaphylaxis rare.

  9. If abdominal pain does not improve, another diagnosis should be considered.

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