Eculizumab to Enable Renal Transplantation in Patients With History of Catastrophic Antiphospholipid Antibody Syndrome

Catastrophic Antiphospholipid Antibody Syndrome (CAPS) is a rare condition in which
life-threatening blood clots form in multiple organs simultaneously and can lead to
multi-organ system failure and death. The causes of CAPS are not entirely understood, but
CAPS episodes are often triggered by stressful events such as infections, surgery, or trauma.
For patients who survive an episode of CAPS, permanent kidney failure is not uncommon because
the kidneys are the organ system most frequently affected in CAPS. Kidney transplantation is
the treatment of choice for end-stage kidney disease, but patients with a history of CAPS are
exceptionally high-risk kidney transplant recipients because the chance that surgery itself
could trigger a life-threatening or transplant-threatening episode of CAPS is significant. As
a result, patients with CAPS are not generally considered candidates for transplantation.
Despite this, these patients have a severely decreased life-expectancy on dialysis and their
long-term survival and quality of life would be greatly increased by a successful kidney
transplant. In this trial, a drug called eculizumab will be tested for its ability to prevent
CAPS after kidney transplantation in patients with a prior history of CAPS. Eculizumab is an
inhibitor of the complement system, which is believed to be important in generating the
inflammatory environment that leads to diffuse clotting of blood vessels in CAPS. The
investigators hypothesize that by blocking the complement cascade using eculizumab, in
conjunction with blocking the coagulation system, that kidney transplantation can be safely
and successfully performed in patients with a history of CAPS.