Bilateral Hydronephrosis due to Rosai-Dorfman Disease

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Introduction: Rosai-Dorfman Disease (RDD) is a rare histiocytic proliferative disorder, with approximately 100 cases diagnosed each year in the United States. Typically it presents as nodal and cutaneous disease, but kidneys can be affected in 4% of cases. Case Description: We present a case of a 78-year-old man who was admitted with osteomyelitis and acute kidney injury. His serum creatinine was elevated to 2.62 mg/dL from baseline of 0.67 mg/dL ten weeks earlier. Retroperitoneal ultrasound showed bilateral hydronephrosis. Further imaging showed obstruction at the level of ureteropelvic junction due to bilateral masses: left 7.0 x 6.1 cm and right 5.7 x 5.2 cm, that were solely enhancing on PET-CT (Figure 1). Biopsy of the right parapelvic mass showed atypical histiocytic and paraplasmic infiltrate consistent with Rosai-Dorfman Disease. Bone culture showed Enterobacter cloacae and patient was started on antibiotics. ANA was detected at 1:320 but C3, C4, and dsDNA were normal and there were no other manifestations to indicate lupus or lupus nephritis. Work-up for other infectious or autoimmune secondary causes was negative. Treatment strategies of this heterogeneous disease vary from surgical resection or debulking, corticosteroids to chemotherapy or radiotherapy. Given ongoing osteomyelitis and further indolent course of his RDD, this patient was managed conservatively with bilateral ureteral stents, and his creatinine improved to 1.4 mg/dL. Discussion: This is a very rare case of Rosai-Dorfman Disease isolated to bilateral kidneys. Ongoing infection hindered use immunosuppression in this patient. This case brings to light a rare disorder and underscores the importance of renal imaging when evaluating acute kidney injury.A. MRI with contrast showing bilateral peripelvic masses.B. PET-CT.

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