Case Report: Atypical Presentation of Idiopathic Retroperitoneal Fibrosis

No standard criteria exist for the classification of idiopathic RPF, and specific diagnostic biomarkers are not available. The lack of significant hydronephrosis in obstructive nephropathy can be misleading and may delay the pursuit of additional investigations. The one-week course of prednisone this patient received for Bell’s palsy might have contributed to further delays in establishing her diagnosis of RPF.

Remission rates following treatment with corticosteroids vary from 75% to 95%; however, idiopathic RPF is a chronic relapsing disorder and even after successful treatment, the relapse rate may be as high as 72%.⁹ This warrants close monitoring and follow up over a long period of time after the initial presentation.

In Sum

Although rare, RPF should be considered in the differential diagnosis of patients presenting with acute kidney injury secondary to obstructive uropathy with periaortic masses seen on imaging. Secondary causes should be excluded. The diagnosis of RPF can be made radiologically, without need for a biopsy.

Despite its chronic relapsing course, idiopathic RPF has good renal outcomes and progression to end-stage renal disease is rare with prompt treatment. 

Roshniben Patel, MD, is a hospitalist at Ascension Saint Agnes Hospital, Baltimore.

Simon Go, MD, is a chief resident in internal medicine at Ascension Saint Agnes Hospital, Baltimore.

Akhila Mohan, MD, is a third-year resident in internal medicine at Ascension Saint Agnes Hospital, Baltimore.

Maria Pardi, MD, is the associate program director of, and an academic hospitalist in, the internal medicine residency program at Ascension Saint Agnes Hospital, Baltimore.

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