School of Medicine and Health Sciences Poster Presentations

Title

An Orphan Disease – Idiopathic Retroperitoneal Fibrosis

Poster Number

274

Document Type

Poster

Status

Medical Student

Abstract Category

Immunology/Infectious Diseases

Keywords

Idiopathic Retroperitoneal Fibrosis, Inflammation, Steroids

Publication Date

Spring 2018

Abstract

Idiopathic Retroperitoneal Fibrosis (IRF) is a rare disorder in which there is a fibrous and inflammatory reaction that occurs in the retroperitoneal space. Patients can present with a wide variety of symptoms depending on the specific location where inflammation occurs and due to this, diagnosis and treatment can often be delayed. Additionally, IRF has yet to be claimed by an Internal Medicine subspecialty which can further complicate the management of these patients. Understanding the presentation and management of patients with IRF is therefore useful. A 69-year-old man with a history of hyperlipidemia, hypertension, heart failure with reduced ejection fraction, and mild cognitive impairment presented to an outside hospital (OSH) with bilateral flank, abdominal, and testicular pain and was found to be in acute renal failure with creatinine of 3.5 (baseline 1.0). CT abdomen and pelvis were performed which showed a large retroperitoneal fibrotic mass encroaching on both ureters, distal abdominal aorta, and common iliac arteries. Urology was consulted for decompression to improve the acute renal failure secondary to obstruction. J stent was placed successfully in the left kidney however due to the fibrotic nature of the mass and its proximity to the right ureter, J stent placement was unsuccessful on the right side. Subsequently, Interventional Radiology (IR) proceeded with percutaneous nephrostomy on the right side. The patient was ultimately transferred to our facility for higher level of care and further diagnostic workup with tissue biopsy to differentiate between IRF and malignancy. Due to the location of the fibrosis near vasculature, there was concern for the risk of vessel penetration during biopsy. Further imaging was performed including a PET scan which did not show any hypermetabolic areas which was favorable for the diagnosis of IRF. Multiple specialties including Nephrology, Urology, General Surgery, Interventional Radiology, and Hematology participated in the conversation regarding the role of tissue biopsy in confirming the diagnosis. However, it was ultimately decided not to pursue a high-risk procedure and instead proceed with treatment of presumable IRF. Treatment with steroids – 80mg daily for 3 months – was initiated almost one month after the patient’s initial presentation at the OSH. Hematology decided to follow the patient as an outpatient. Presentation of IRF can vary between patients and because it is adopted by different specialists based on presenting symptoms, management can vary. This is a case of a patient with presumable IRF with delay in treatment due to pending diagnostic workup. Given the lack of management guidelines for IRF, a shared decision-making process was pursued between the patient, primary team, and multiple subspecialists.

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An Orphan Disease – Idiopathic Retroperitoneal Fibrosis

Idiopathic Retroperitoneal Fibrosis (IRF) is a rare disorder in which there is a fibrous and inflammatory reaction that occurs in the retroperitoneal space. Patients can present with a wide variety of symptoms depending on the specific location where inflammation occurs and due to this, diagnosis and treatment can often be delayed. Additionally, IRF has yet to be claimed by an Internal Medicine subspecialty which can further complicate the management of these patients. Understanding the presentation and management of patients with IRF is therefore useful. A 69-year-old man with a history of hyperlipidemia, hypertension, heart failure with reduced ejection fraction, and mild cognitive impairment presented to an outside hospital (OSH) with bilateral flank, abdominal, and testicular pain and was found to be in acute renal failure with creatinine of 3.5 (baseline 1.0). CT abdomen and pelvis were performed which showed a large retroperitoneal fibrotic mass encroaching on both ureters, distal abdominal aorta, and common iliac arteries. Urology was consulted for decompression to improve the acute renal failure secondary to obstruction. J stent was placed successfully in the left kidney however due to the fibrotic nature of the mass and its proximity to the right ureter, J stent placement was unsuccessful on the right side. Subsequently, Interventional Radiology (IR) proceeded with percutaneous nephrostomy on the right side. The patient was ultimately transferred to our facility for higher level of care and further diagnostic workup with tissue biopsy to differentiate between IRF and malignancy. Due to the location of the fibrosis near vasculature, there was concern for the risk of vessel penetration during biopsy. Further imaging was performed including a PET scan which did not show any hypermetabolic areas which was favorable for the diagnosis of IRF. Multiple specialties including Nephrology, Urology, General Surgery, Interventional Radiology, and Hematology participated in the conversation regarding the role of tissue biopsy in confirming the diagnosis. However, it was ultimately decided not to pursue a high-risk procedure and instead proceed with treatment of presumable IRF. Treatment with steroids – 80mg daily for 3 months – was initiated almost one month after the patient’s initial presentation at the OSH. Hematology decided to follow the patient as an outpatient. Presentation of IRF can vary between patients and because it is adopted by different specialists based on presenting symptoms, management can vary. This is a case of a patient with presumable IRF with delay in treatment due to pending diagnostic workup. Given the lack of management guidelines for IRF, a shared decision-making process was pursued between the patient, primary team, and multiple subspecialists.