Clinical characteristics and favorable treatment responses of recurrent focal segmental glomerulosclerosis or steroid-resistant nephrotic syndrome in children after kidney transplantation.

Dharnidharka, Vikas R; Scobell, Rebecca R; Kallash, Mahmoud; Davies, Amy J Goodwin; Marchesani, Nicole; Maltenfort, Mitchell G; Walther, Leslie; Kelton, Megan; Bock, Margret; Blanchette, Eliza; Stone, Hillarey K; Gluck, Caroline; Hullekes, Frank; Riella, Leonardo V; Smoyer, William E; Mitsnefes, Mark; Dixon, Bradley P; Flynn, Joseph T; Somers, Michael J G; Forrest, Christopher B; Furth, Susan; Denburg, Michelle R

Clinical characteristics and favorable treatment responses of recurrent focal segmental glomerulosclerosis or steroid-resistant nephrotic syndrome in children after kidney transplantation.

Dharnidharka, Vikas R; Scobell, Rebecca R; Kallash, Mahmoud; Davies, Amy J Goodwin; Marchesani, Nicole; Maltenfort, Mitchell G; Walther, Leslie; Kelton, Megan; Bock, Margret; Blanchette, Eliza; Stone, Hillarey K; Gluck, Caroline; Hullekes, Frank; Riella, Leonardo V; Smoyer, William E; Mitsnefes, Mark; Dixon, Bradley P; Flynn, Joseph T; Somers, Michael J G; Forrest, Christopher B; Furth, Susan; Denburg, Michelle R.

Dharnidharka VR; Washington University School of Medicine and St. Louis Children's Hospital, Room NWT 10-119, CB 8116, 660 South Euclid Avenue, St. Louis, MO, 63110, USA. vikasD@wustl.edu.
Scobell RR; Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Kallash M; Department of Pediatrics, The Research Institute at Nationwide Children's Hospital, The Ohio State University, Columbus, OH, USA.
Davies AJG; Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Marchesani N; Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Maltenfort MG; Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Walther L; Washington University School of Medicine and St. Louis Children's Hospital, Room NWT 10-119, CB 8116, 660 South Euclid Avenue, St. Louis, MO, 63110, USA.
Kelton M; Department of Pediatrics, University of Washington School of Medicine, Seattle, WA, USA.
Bock M; Renal Section, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO, USA.
Blanchette E; Renal Section, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO, USA.
Stone HK; Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
Gluck C; Nemours Children's Health, Jacksonville, FL, USA.
Hullekes F; Massachusetts General Hospital, Boston, MA, USA.
Riella LV; Massachusetts General Hospital, Boston, MA, USA.
Smoyer WE; Department of Pediatrics, The Research Institute at Nationwide Children's Hospital, The Ohio State University, Columbus, OH, USA.
Mitsnefes M; Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
Dixon BP; Renal Section, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO, USA.
Flynn JT; Department of Pediatrics, University of Washington School of Medicine, Seattle, WA, USA.
Somers MJG; Boston Children's Hospital, Boston, MA, USA.
Forrest CB; Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Furth S; Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Denburg MR; Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Pediatr Nephrol ; 2024 Jul 13.

Article en En | MEDLINE | ID: mdl-39001911

ABSTRACT

ABSTRACT

BACKGROUND:

Recurrence of focal segmental glomerulosclerosis (FSGS) or steroid-resistant nephrotic syndrome (SRNS) after kidney transplant leads to significant morbidity and potentially earlier allograft loss. To date however, reported rates, risk factors and treatment outcomes have varied widely.

METHODS:

We applied computational phenotypes to a multicenter aggregation of electronic health records data from 7 large pediatric health systems in the USA, to identify recurrence rates, risk factors, and treatment outcomes. We refined the data collection by chart review.

RESULTS:

From > 7 million patients, we compared children with primary FSGS/SRNS who received a kidney transplant between 2009 and 2020 and who either developed recurrence (n = 67/165; 40.6%) or did not (n = 98/165). Serum albumin level at time of transplant was significantly lower and recipient HLA DR7 presence was significantly higher in the recurrence group. By 36 months post-transplant, complete remission occurred in 58.2% and partial remission in 17.9%. Through 6 years post-transplant, no remission after recurrence was associated with an increased risk of allograft loss over time (p < 0.0001), but any remission showed similar allograft survival and function decline to those with no recurrence. Since treatments were used in non-random fashion, using spline curves and multivariable non-linear analyses, complete + partial remission chance was significantly higher with greater plasmapheresis sessions, CTLA4-Ig doses or LDL-apheresis sessions. Only treatment with anti-CD20, CTLA4-Ig agents, or LDL-apheresis sessions were associated with complete remission. Excluding 25 patients with mutations did not significantly change our results.