Objective: To describe the clinical features and treatment response of Rosai-Dorfman disease (RDD) and Erdheim-Chester disease (ECD) with kidney involvement.

Methods: We conducted a retrospective analysis of patients with RDD and ECD who had kidney involvement between January 2005 and December 2023. We evaluated the kidney response based on kidney function, computed tomography (CT) response and metabolic responses.

Results: A total of 4 RDD patients and 44 ECD patients were included, with a median age of 58 (36-68) and 51(18-66) years, respectively. None of the RDD patients had kidney-associated symptoms, whereas 12 (27.3%) ECD patients presented with lower extremity edema. Regarding baseline kidney function, the median eGFR of RDD and ECD was 80.5 (range: 63-125) and 100 (range: 22-133) ml/min/1.73 m², respectively. Renal mass was the most frequent characteristic of RDD, accounting for 100%, while ECD patients showed a high perirenal infiltration rate (68.2%). Among the 4 RDD patients, one (25.0%) achieved eGFR improvement and two (50.0%) achieved CT response and PET-CT response. In 34 ECD patients evaluable, the percentage of eGFR improvement, CT response and PET-CT response was 14.7%, 5.9% and 52.9%. The median follow-up time was 27.0 months (range: 10.1-52.9 months) for RDD and 53.0 months (range: 3.1-105.3 months) for ECD. The 5-year OS was 66.7% in RDD patients and 81.8% in ECD patients. The median PFS for patients with RDD was 18.3 months, while for those with ECD, it was 59.4 months.

Conclusion: We pioneered the detailed description of kidney involvement characteristics and treatment responses in RDD and ECD. RDD tended to be featured with occupying mass, while ECD is characterized by soft tissue encircling, which is less likely to regress after treatment. Patients with ECD may experience irreversible kidney dysfunction, indicating the critical need for early diagnosis and timely treatment.

Disclosures

No relevant conflicts of interest to declare.

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2024
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