ASN's Mission

ASN leads the fight to prevent, treat, and cure kidney diseases throughout the world by educating health professionals and scientists, advancing research and innovation, communicating new knowledge, and advocating for the highest quality care for patients.

learn more

Contact ASN

1401 H St, NW, Ste 900, Washington, DC 20005

email@asn-online.org

202-640-4660

The Latest on Twitter

Kidney Week

Abstract: PO2562

Living Donor-Derived APOL1-Associated Collapsing FSGS in a Kidney Transplant Recipient

Session Information

Category: Transplantation

  • 1902 Transplantation: Clinical

Authors

  • Agha, Irfan, Dallas Renal Group, Dallas, Texas, United States
  • Ilahe, Amna, Dallas Renal Group, Dallas, Texas, United States
  • Simon, Silvi, Dallas Renal Group, Dallas, Texas, United States
  • Nadella, Rama, Dallas Renal Group, Dallas, Texas, United States
  • Mahbod, Diana, Dallas Renal Group, Dallas, Texas, United States
  • Dickerman, Richard, Methodist Dallas Medical Center, Dallas, Texas, United States
Introduction

Homozygous high risk APOL-1 mutations in donors can drive disease in recipients.
We present a living kidney recipient with de-novo collapsing FSGS. His donor was homozygous for high risk mutations.

Case Description

47 YO AA male with CKD due to biopsy proven diabetic nephropathy s/p LUKT in May 2018. Got thymoglobulin (5 mg/kg) with tacrolimus, mycophenolate sodium and prednisone. High risk for CMV (D+ / R-). EBV IgG +.
Donor was his 35 years old AA wife. We were not testing APOL-1 on AA donors at the time.

At month 6: Cr 1.4 mg/dl, Ur Pr/Cr of 0.22 g/g. Valganciclovir stopped. Two weeks later, Ur Pr/Cr 2.92 g/g. CMV PCR + 276 copies / ml and EBV PCR + 492 copies /ml. PCR for Parvo Virus B-19 as well as DSA negative.
Given steroids, plasmapheresis (X3) and IV ganciclovir.
Biopsy C/W collapsing FSGS. IF negative, including CMV and C4d.
Donor tested for and found to be homozygous for APOL1 G1 mutations confirming donor derived APOL -1 gene associated collapsing FSGS, presenting as an acute diffuse podocytopathy.

Trends in his renal function, CMV, EBV and proteinuria are tabulated in Figure 1.

Treated with RAAS blockade, antivirals & immunosuppression. He cleared his CMV Viremia promptly. However, EBV viremia worsened. Eventually, in May 2019, received Rituximab with resolution of EBV viremia.
He is 18 months out from presentation. Cr is in the 2 mg/dl range with persistent proteinuria.

The life of the allograft will be significantly curtailed.
His donor is doing well with stable kidney function and no proteinuria.

Discussion

APOL-1 mutations place recipients of these kidneys at risk for adverse outcomes. Few instances of APOL-1 mutation driven kidney disease in recipients from living donors are documented.
This presentation supports the two-hit hypothesis - The high interferon state due to CMV and EBV viremia precipitated APOl-1 associated FSGS in this kidney with homozygous high risk mutations.
APOL-1 screening in AA donors, especially young donors, should be considered for risk stratification and counseling.

Clinical Course