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Kidney Week

Abstract: SA-PO364

Thrombotic Microangiopathy as the Presenting Feature of Newly Diagnosed HIV Infection Treated with Eculizumab

Session Information

Category: Trainee Case Report

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Yunes, Milagros, Montefiore Medical Center, Bronx, New York, United States
  • Pullman, James M., Montefiore Medical Center, Bronx, New York, United States
  • Fisher, Molly, Montefiore Medical Center, Bronx, New York, United States
Introduction

Thrombotic microangiopathy (TMA) is a known complication of HIV infection. HIV infection precipitates endothelial injury and has been associated with increased complement activation. It may also trigger the onset of atypical HUS in patients with genetic predisposition. We present a case of severe kidney failure due to TMA in the setting of newly diagnosed HIV infection which was successfully treated with eculizumab.

Case Description

58-year-old Hispanic male with history of hypertension presented with 1 month of diarrhea, lower extremity edema and oliguria. Initial labs revealed a serum creatinine 9.0 mg/dL, albumin <2.0 g/dL, urine protein/creatinine 8 g/g, platelet count 103,000, hemoglobin 7.9 g/dL, LDH 619 U/L and schistocytes on peripheral smear. Workup revealed newly diagnosed HIV/AIDS with a CD4 count of 40 cells/uL, viral load of 124,000 copies and coinfection with hepatitis B. Kidney biopsy demonstrated chronic active TMA with focally positive c4d staining of the glomerular capillaries and acute tubular necrosis. ADAMTS13 was normal and stool Shiga toxin was negative. He was found to have CMV viremia but immunostaining for CMV on kidney biopsy was negative. Genetic testing for complement abnormalities revealed a complement factor H receptor 5 variant of unknown significance but was otherwise negative. The patient was dialysis dependent at presentation. He was initiated on antiretroviral therapy and started on eculizumab infusions every 2 weeks for treatment of HIV-associated TMA. Although his CD4 count remained low at 70, his HIV viral load became undetectable, his hematologic parameters improved and creatinine clearance improved to 25 mL/min leading to discontinuation of dialysis after 2 months.

Discussion

In the modern era of antiretroviral therapy, TMA is a rare complication of HIV infection. Because uncontrolled HIV infection has been associated with increased complement activation, we hypothesized that HIV infection triggered dysregulation of complement activity in our patient and that treatment with eculizumab would be beneficial. Although our patient did not have a common gene mutation associated with atypical HUS, treatment with eculizumab in conjunction with ART resulted in hematologic remission as well as improvement in kidney function and discontinuation of dialysis.