A Study to Evaluate the Benefit of RUCONEST® in Subjects Who Experience ADRs Related to IVIG Infusions

This is a single-site, pilot study conducted in the US to determine the benefit of human
C1-esterase inhibitor [recombinant] (C1-INH-R) therapy to ameliorate ADRs in subjects
receiving IVIG therapy experience ADRs post-infusion. Subjects who are currently receiving
IVIG for immunodeficiency or neurologic conditions and experience ADRs will be enrolled.

In part 1, subjects will continue to receive IVIG for two infusions. Complement proteins will
be measured pre- and post-infusion. Quality of life and other questionnaires will be
administered.

In part 2, subjects will receive C1-INH-R infusion prior to receiving IVIG infusion for three
infusions. Complement proteins will be measured pre- and post- infusion. Quality of life and
other questionnaires will be administered.

Inclusion Criteria:

- Age 18 years and older experiencing ADRs related to IVIG infusions

- Stable dose of IVIG for 3 months

- Willing to comply with all aspects of the protocol, including blood draws

- Female patients of childbearing potential who are sexually active must be willing to
use an acceptable form of contraception. Acceptable forms of contraception are defined
as those with a failure rate < 1% when properly applied and include: a combination
oral pill, some intra-uterine devices, and a sterilized partner in a stable
relationship. Female patients must not be pregnant, planning to become pregnant, or be
actively breastfeeding through the entire study period.

Exclusion Criteria:

- Receiving treatment for HAE, either prophylactic or acute therapy

- Patients with medical history of allergy to rabbits or rabbit-derived products
(including rhC1INH)

- Patients who are pregnant, or breastfeeding, or are currently intending to become
pregnant.

- Patients who, in the investigator's opinion, might not be suitable for the trial