BMC Nephrology (Nov 2021)

Clinical outcomes and temporal trends of immunological and non-immunological rare diseases in adult kidney transplant

  • Ester Gallo,
  • Silvia Mingozzi,
  • Alberto Mella,
  • Fabrizio Fop,
  • Roberto Presta,
  • Manuel Burdese,
  • Elena Boaglio,
  • Maria Cristina Torazza,
  • Roberta Giraudi,
  • Gianluca Leonardi,
  • Antonio Lavacca,
  • Paolo Gontero,
  • Omidreza Sedigh,
  • Andrea Bosio,
  • Aldo Verri,
  • Caterina Dolla,
  • Luigi Biancone

DOI
https://doi.org/10.1186/s12882-021-02571-z
Journal volume & issue
Vol. 22, no. 1
pp. 1 – 13

Abstract

Read online

Abstract Background Rare diseases (RDs) encompass many difficult-to-treat conditions with different characteristics often associated with end-stage renal disease (ESRD). However, data about transplant outcomes in adult patients are still lacking and limited to case reports/case series without differentiation between immunological/non-immunological RDs. Methods Retrospective analysis among all adult kidney transplanted patients (KTs) with RDs (RDsKT group) performed in our high-volume transplantation center between 2005 and 2016. RDs were classified according to the Orphanet code system differentiating between immunological and non-immunological diseases, also comparing clinical outcomes and temporal trends to a control population without RDs (nRDsKT). Results Among 1381 KTs, 350 patients (25.3%) were affected by RDs (RDsKTs). During a f/up > 5 years [median 7.9 years (4.8–11.1)], kidney function and graft/patient survival did not differ from nRDsKTs. Considering all post-transplant complications, RDsKTs (including, by definition, patients with primary glomerulopathy except on IgA nephropathy) have more recurrent and de-novo glomerulonephritis (14.6% vs. 9.6% in nRDsKTs; p = 0.05), similar rates of de-novo cancers, post-transplant diabetes, dysmetabolism, hematologic disorders, urologic/vascular problems, and lower infectious episodes than nRDsKTs (63.7% vs 72.7%; p = 0.013). Additional stratification for immunological and non-immunological RDsKTs or transplantation periods (before/after 2010) showed no differences or temporal trends between groups. Conclusions Kidney transplant centers are deeply involved in RDs management. Despite their high-complex profile, both immunological and non-immunological RDsKTs experienced favorable patients’ and graft survival.

Keywords