Abstract: FR-PO132

Successful Treatment of Severe AKI Caused by Catastrophic Anti-Phospholipid Syndrome (CAPS) Associated Thrombotic Microangiopathy (TMA) by Anti-C5 Monoclonal Antibody

Session Information

Category: Acute Kidney Injury

  • 003 AKI: Clinical and Translational

Authors

  • Gupta, Sanjeev, Westchester Medical Center, Valhalla, New York, United States
  • Chander, Praveen N., New York Medical College , Valhalla, New York, United States
  • Papanagnou, Anastasios, Westchester Medical Center, Valhalla, New York, United States
  • Katchi, Tasleem, Westchester Medical Center, Valhalla, New York, United States
  • Chugh, Savneek S., New York Medical College , Valhalla, New York, United States
Background

CAPS is a severe form of the anti-phospholipid syndrome, characterized by arterial and/or venous thrombosis leading to multi-organ failure, occurring over a short period of time. Steroids, IVIG, and plasmapheresis have long been the standard of care. Recently, the role of complement inhibition in CAPS has been explored. We present a very rare case of refractory CAPS successfully treated with Eculizumab (Ecu) which is a monoclonal antibody against complement protein C5.

Methods

A 65 years old male with a history of positive antiphospholipid antibodies (APA) presented with intermittent abdominal pain for 5 months and altered mentation. Physical examination highlighted a malar rash and bluish discoloration of the left 5th toe. Laboratory workup revealed anemia (Hgb-7.5g/DL), thrombocytopenia (platelet- 106,000) and AKI (SCr 7.53), positive ANA, anti-dsDNA Ab, elevated lupus anticoagulant (LA- 1.69:1), anti-cardiolipin IgM (33), anti-β2-glycoprotein (22), low C3 & C4 (52, <5) and schistocytes on peripheral blood smear. Renal biopsy showed typical findings of TMA. ADAMTS13 activity was 25 which ruled out thrombotic thrombocytopenic purpura. Given multiple organ involvements within a week, TMA on renal biopsy and positive APA, a diagnosis of CAPS was made and the patient was started on steroids, plasmapheresis, IVIG and hemodialysis (HD). Despite adequate immunosuppression, LA titers remained elevated, and Rituximab was started. The patient subsequently failed treatment and remained on HD for 3 months. Because of ongoing TMA, Ecu was started which resulted in improvement of thrombocytopenia and urine output. The patient subsequently recovered his renal function and HD was discontinued 2 months after Ecu initiation.

Conclusion

Therapeutic options for refractory cases are limited. Rituximab is the next line treatment but, our patient failed that as well. Based on few case reports patient was started on Ecu and responded well. Ecu is a potent terminal complement blockade, which appears to be equally effective in CAPS induced TMA as in aHUS. In conclusion, complement blockade can be a useful mode of therapy in CAPS even in cases that have failed standard treatment and after months of organ failure.