Kidney Transplantation in Patients With Erdheim-Chester Disease

Abstract

Erdheim-Chester disease (ECD) is a rare histiocytosis driven by somatic mutations involving genes of the MAPK pathway (e.g., BRAFV600E).1 Bilateral perirenal and retroperitoneal involvement occurs in approximately 70% of cases and typically consists of infiltration of the perirenal space (“hairy kidney” appearance), the pelvis, the proximal ureter, and the renal vascular peduncle2 (Figure 1a–f). It is often asymptomatic but may cause abdominal or lumbar pain and renovascular hypertension. Unrecognized long-standing perirenal infiltration can lead to obstructive or ischemic nephropathy, ultimately causing chronic kidney disease and, in some cases, kidney failure.

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Cite

BibTeX RIS

BibTeX

@article{palazzini2026kidney,
  title = {{Kidney Transplantation in Patients With Erdheim-Chester Disease}},
  author = {Giulia Palazzini and Francesco Pegoraro and Jean-François Emile and Sanjeev Akkina and Elisa Buti and Anna Perrone and Corrado Campochiaro and Lorenzo Dagna and Edoardo La Porta and Decimo Silvio Chiarenza and Anna J. Peired and Achille Aouba and Raphaële Renard-Penna and Julien Haroche and Augusto Vaglio},
  journal = {Kidney International Reports},
  year = {2026},
  month = nov,
  doi = {10.1016/j.ekir.2025.103763},
  url = {https://hal.science/hal-05849907v1},
  note = {CC BY},
}

RIS

TY  - JOUR
TI  - Kidney Transplantation in Patients With Erdheim-Chester Disease
AU  - Giulia Palazzini
AU  - Francesco Pegoraro
AU  - Jean-François Emile
AU  - Sanjeev Akkina
AU  - Elisa Buti
AU  - Anna Perrone
AU  - Corrado Campochiaro
AU  - Lorenzo Dagna
AU  - Edoardo La Porta
AU  - Decimo Silvio Chiarenza
AU  - Anna J. Peired
AU  - Achille Aouba
AU  - Raphaële Renard-Penna
AU  - Julien Haroche
AU  - Augusto Vaglio
PY  - 2026
DA  - 2026/11/03
JO  - Kidney International Reports
DO  - 10.1016/j.ekir.2025.103763
UR  - https://hal.science/hal-05849907v1
AB  - Erdheim-Chester disease (ECD) is a rare histiocytosis driven by somatic mutations involving genes of the MAPK pathway (e.g., BRAFV600E).1 Bilateral perirenal and retroperitoneal involvement occurs in approximately 70% of cases and typically consists of infiltration of the perirenal space (“hairy kidney” appearance), the pelvis, the proximal ureter, and the renal vascular peduncle2 (Figure 1a–f). It is often asymptomatic but may cause abdominal or lumbar pain and renovascular hypertension. Unrecognized long-standing perirenal infiltration can lead to obstructive or ischemic nephropathy, ultimately causing chronic kidney disease and, in some cases, kidney failure.
SN  - 2468-0249
ER  -

Abstract

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