TY - JOUR
T1 - Kidney Transplantation in Childhood-Onset Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
T2 - Long-Term Outcomes and Prognostic Factors
AU - CIBREO study group
AU - Trivioli, Giorgio
AU - Allinovi, Marco
AU - Di Marcantonio, Elio
AU - Jawa, Natasha A
AU - Trivelli, Antonella
AU - Yang, Jing
AU - Grenda, Ryszard
AU - Rubik, Jacek
AU - Mohammad, Aladdin J
AU - Testa, Sara
AU - Jahnukainen, Timo
AU - Gulhan, Bora
AU - Topaloglu, Rezan
AU - Puéchal, Xavier
AU - Kosałka-Węgiel, Joanna
AU - Dursun, Ismail
AU - Guzzo, Isabella
AU - Pasini, Andrea
AU - Kostik, Mikhail M
AU - Oni, Louise
AU - Buti, Elisa
AU - Becherucci, Francesca
AU - Palazzini, Giulia
AU - Moroni, Gabriella
AU - Ozen, Seza
AU - Laurent, Audrey
AU - Harambat, Jerome
AU - Dossier, Claire
AU - Marks, Stephen D
AU - Bruchfeld, Annette
AU - Saleem, Moin
AU - al, et
AU - Vaglio, Augusto
N1 - Copyright © 2026 by the American Society of Nephrology.
PY - 2026/3/1
Y1 - 2026/3/1
N2 - Key Points
Among 72 patients with childhood-onset ANCA-associated vasculitis, kidney transplant survival was good (86%).ANCA-associated
vasculitis relapse occurred in 8 patients (11%), a median of 71 months
after transplantation and resulted in graft failure in only one case.Positive
ANCA at the time of transplantation did not predict graft failure but
was associated with a higher risk of relapse and worse graft function.
Background
ANCA-associated vasculitis (AAV) is rare in children, and
results in kidney failure in up to one third of cases. There is very
limited knowledge on kidney transplantation in childhood-onset AAV. We
assessed kidney transplantation outcomes and prognostic factors in a
multicenter cohort of patients with childhood-onset AAV.
Methods
Patients diagnosed with AAV during childhood (≤18 years)
who received a kidney transplant were included in this retrospective
study. We determined patient and graft survival, rates of chronic graft
dysfunction (defined as eGFR <60 ml/min per 1.73 m2
for ≥3 months) and AAV relapse, and assessed determinants of outcome
with logistic regression models. Patients were matched 1:2 for age, sex,
and era of transplantation with non-AAV recipients from the Hospital
for Sick Children in Toronto, Canada, and their graft survival was
compared.
Results
We included 72 patients, of whom 53 (74%) had microscopic
polyangiitis and 19 (26%) granulomatosis with polyangiitis. Their median
age (interquartile range at the time of diagnosis and transplantation
was 12 (9–14) and 14 (12–16) years, respectively. After a median
post-transplant follow-up of 53 months (interquartile range, 25–97), 70
patients (97%) were alive, 62 (86%) had a functioning graft, 28 (39%)
had developed chronic graft dysfunction, and 8 (11%) had experienced AAV
relapse. Graft survival was comparable between AAV and non-AAV
recipients. Acute rejection was the only independent predictor of graft
failure (hazard ratio [HR], 12.11; 95% confidence interval [CI], 1.19 to
122.49). Positive ANCA at the time of transplantation was significantly
associated with a chronic graft dysfunction (HR, 4.16; 95% CI, 1.71 to
10.13) and AAV relapse (HR, 23.1; 95% CI, 2.67 to 200.28).
Conclusions
Patients with childhood-onset AAV show good overall and
graft survival after kidney transplantation and a low rate of
post-transplant relapse. Further studies are warranted to confirm
whether positive ANCA at the time of transplantation is associated with
poorer graft outcomes.
AB - Key Points
Among 72 patients with childhood-onset ANCA-associated vasculitis, kidney transplant survival was good (86%).ANCA-associated
vasculitis relapse occurred in 8 patients (11%), a median of 71 months
after transplantation and resulted in graft failure in only one case.Positive
ANCA at the time of transplantation did not predict graft failure but
was associated with a higher risk of relapse and worse graft function.
Background
ANCA-associated vasculitis (AAV) is rare in children, and
results in kidney failure in up to one third of cases. There is very
limited knowledge on kidney transplantation in childhood-onset AAV. We
assessed kidney transplantation outcomes and prognostic factors in a
multicenter cohort of patients with childhood-onset AAV.
Methods
Patients diagnosed with AAV during childhood (≤18 years)
who received a kidney transplant were included in this retrospective
study. We determined patient and graft survival, rates of chronic graft
dysfunction (defined as eGFR <60 ml/min per 1.73 m2
for ≥3 months) and AAV relapse, and assessed determinants of outcome
with logistic regression models. Patients were matched 1:2 for age, sex,
and era of transplantation with non-AAV recipients from the Hospital
for Sick Children in Toronto, Canada, and their graft survival was
compared.
Results
We included 72 patients, of whom 53 (74%) had microscopic
polyangiitis and 19 (26%) granulomatosis with polyangiitis. Their median
age (interquartile range at the time of diagnosis and transplantation
was 12 (9–14) and 14 (12–16) years, respectively. After a median
post-transplant follow-up of 53 months (interquartile range, 25–97), 70
patients (97%) were alive, 62 (86%) had a functioning graft, 28 (39%)
had developed chronic graft dysfunction, and 8 (11%) had experienced AAV
relapse. Graft survival was comparable between AAV and non-AAV
recipients. Acute rejection was the only independent predictor of graft
failure (hazard ratio [HR], 12.11; 95% confidence interval [CI], 1.19 to
122.49). Positive ANCA at the time of transplantation was significantly
associated with a chronic graft dysfunction (HR, 4.16; 95% CI, 1.71 to
10.13) and AAV relapse (HR, 23.1; 95% CI, 2.67 to 200.28).
Conclusions
Patients with childhood-onset AAV show good overall and
graft survival after kidney transplantation and a low rate of
post-transplant relapse. Further studies are warranted to confirm
whether positive ANCA at the time of transplantation is associated with
poorer graft outcomes.
U2 - 10.2215/CJN.0000000936
DO - 10.2215/CJN.0000000936
M3 - Article (Academic Journal)
C2 - 41505217
SN - 1555-9041
VL - 21
SP - 480
EP - 493
JO - Clinical Journal of the American Society of Nephrology
JF - Clinical Journal of the American Society of Nephrology
IS - 3
ER -