Subcutaneous Use of the Plasma Derived C1 Inhibitor Berinert in a Complicated Hereditary Angioedema Case
Monday, March 7, 2016
South Exhibit Hall H (Convention Center)
Amin S. Kanani, MD
Rationale: Hereditary angioedema (HAE) is caused by an inherited deficiency (type 1) or dysfunction (type2) of the plasma protein C1 inhibitor (C1INH). Here we present a complicated HAE case requiring subcutaneous use of the C1INH, Berinert for prophylaxis.

Methods: Patient was trained to self-administer C1INH subcutaneously.

Results: A 40 year old female, was diagnosed with HAE type 1 at the age of 19.  Since then she has had over 250 ER visits requiring C1INH infusions.  Her initial treatment consisted of prophylactic Danazol which was discontinued after 15 years due to lack of efficacy and increased side effects.  She was then trained on prophylactic use of C1INH intravenous home infusion at 1500 IU Q2days.  Due to poor peripheral venous access she had a central venous catheter (CVC) inserted.  The CVC was however removed due to development of a fibrin sheath and obstructing thrombus. In November 2014 a trial of subcutaneous C1INH infusions was initiated at 4000 IU twice a week. Dose was titrated up to 4000 IU Q2days which maintained reasonable control.  The patient has not had ER visits for HAE attacks since starting the subcutaneous C1INH infusions.  Functional C1INH assay increased from 0.29 (normal 0.7-1.3 U) prior to starting C1INH subcutaneous infusions to 0.76 at 6 months after initiating therapy.

Conclusions: This case demonstrates the efficacy of prophylactic subcutaneous C1INH in an adult patient with HAE, with a reduction in HAE attacks and ER visits, and an improvement in the C1INH functional assay.