Resolution of Catastrophic Anti Phospholipid Antibody Syndrome with Acute Renal Failure after Treatment with Rituximab.

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Abstract Antiphospholipid antibody syndrome (APS) is a thrombophilic disorder defined by the presence of recurrent thrombosis associated with anticardiolipin antibodies (ACA). To date, there is no effdective therapy for this condition. Rituximab, a chimeric monoclonal antibody B Cells has been shown to be effective in autoimmune disorders. Sporadic abstracts have reported the disappearance of ACA after Rituximab treatment. We have successfully treated a patient with primary catastrophic APS and renal failure with Rituximab. Case Report: A thirty seven year old male with history of DVT, ischemic cardiomyopathy and cerebrovascular accident was admitted to the Hospital for the treatment of acute renal failure and thrombocytopenia. Physical examination was significant for cognitive deficits, mild hepatomegaly, generalized livido reticulairs, and acrocyanosis. Laboratory data revealed platelet count of 97000, creatinine 3.4 mg/dl, IgG ACA titer of 43.8 and negative IgM ACA and ANA. He had proteinuria with benign urinary sediment. Kidney biopsy showed thrombotic microangiopathy. Plasmaphersis was initiated and hemodialysis for the rapidly progressing renal failure. He continued his treatment in an outpatient hemodialysis unit while on Coumadin. Four weekly cycles of Rituximab were given as an outpatient. Serial laboratory investigations showed gradual normalization of the platelet count and disappearance of the IgG ACA. Gradually renal function recovered and hemodialysis was discontinued after three months. He remains in remission with no further thrombotic events.Conclusion: Rituximab is promising in the treatment of APS and must be considered in the first;line treatment of catastrophic APS. A clinical trial is necessary to test the feasibility of discontinuing anticoagulation in these patients. PERTINENT LABORATORY DATA DATE HEMOGLOBIN PLATELETS CREATININE ACA TITER 03/21/02 1.4 11/15/03 14.4 97 3.4 11/21/03 12.5 100 6.2 43.8 12/01/03 12.2 222 ON DIALYSIS 16.1 01/01/04 14.1 276 ON DIALYSIS 20.9 03/17/04 14.1 276 ON DIALYSIS 05/05/04 11.3 330 3.3 NEGATIVE

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