Kidney Week

Abstract: TH-PO0070

Kidney Involvement in Rosai-Dorfman Disease: A Rare Case of Acute Tubulointerstitial Nephritis

Session Information

• AKI: Pathogenesis and Disease Mechanisms
  November 06, 2025 | Location: Exhibit Hall, Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Acute Kidney Injury

• 102 AKI: Clinical, Outcomes, and Trials

Authors

• Mashayekhi, Mahtab, Loma Linda University Medical Center, Loma Linda, California, United States

Mahtab Mashayekhi, MD

• Bhullar, Jasjot K., Loma Linda University Medical Center, Loma Linda, California, United States

Jasjot K. Bhullar, MD

• Chen Wongworawat, Yan, Loma Linda University Medical Center, Loma Linda, California, United States

Yan Chen Wongworawat

• Abdi Pour, Amir, Loma Linda University Medical Center, Loma Linda, California, United States

Amir Abdi Pour, MD, FASN

• Norouzi, Sayna, Loma Linda University Medical Center, Loma Linda, California, United States

Sayna Norouzi, MD, FASN

• Narayan, Melin Joe, Loma Linda University Medical Center, Loma Linda, California, United States

Melin Joe Narayan, MD, FASN

Introduction

Rosai-Dorfman-Destombes disease (RDD) is a rare non-Langerhans cell histiocytosis with lymphadenopathy, and has an unknown etiology. In extra-nodal manifestations, kidney involvement is rare, only observed in 4% of cases, and is associated with a poor prognosis. Kidney manifestation usually is a hilar mass, while parenchymal involvement without mass is extremely uncommon.

Case Description

A 50-year-old male with a previous diagnosis of RDD, was referred to the emergency department after a routine follow-up revealed an elevated creatinine. He reported intermittent right-sided flank pain for two weeks and occasional nausea over the previous three days. Past medical history included hypertension, type 2 diabetes mellitus, and alcohol use disorder. On physical examination, he was hemodynamically stable but was noted to have right costovertebral angle tenderness. Labs showed creatinine 3.8 mg/dL (baseline of 1.2 mg/dL, 3 months prior), blood urea nitrogen (BUN) 44 mg/dL, estimated glomerular filtration rate (eGFR)18 mL/min/1.73m², and low C3 and C4 levels. Kidney ultrasound demonstrated bilaterally enlarged kidneys with mild parenchymal heterogeneity.
Kidney biopsy results revealed severe, nearly diffuse tubulointerstitial nephritis (TIN) composed of predominantly histiocytes. Immunohistochemistry showed histiocytes strongly positive for S100, CD68, and CD163, and negative for CD1a, consistent with kidney involvement by RDD (Figure 1).
Treatment with prednisone 60 mg daily was initiated for the patient, and after one month, creatinine decreased to 1.56 mg/dL, and eGFR increased to 53 mL/min/1.73m², followed by a gradual taper of prednisone.

Discussion

Acute TIN associated with RDD is a very rare but serious condition. Monitoring kidney function, early recognition, and histopathologic confirmation of S100/CD68-positive and CD1a-negative histiocytes are essential for timely diagnosis and initiation of therapy to preserve kidney function.

Digital Object Identifier (DOI)

• doi: 10.1681/ASN.202564z0n3nq