Kidney Week

Abstract: TH-PO1042

Exploring the Spectrum of Retroperitoneal Fibrosis

Session Information

• CKD Progression and Complications: Diagnosis, Prognosis, Risk Factors
  November 02, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: CKD (Non-Dialysis)

• 2302 CKD (Non-Dialysis): Clinical, Outcomes, and Trials

Authors

• Srialluri, Nityasree, Johns Hopkins Medicine, Baltimore, Maryland, United States

Nityasree Srialluri,

• Le, Dustin, Johns Hopkins Medicine, Baltimore, Maryland, United States

Dustin Le,

• Molano, Grace A., Johns Hopkins Medicine, Baltimore, Maryland, United States

Grace A. Molano,

• Atta, Mohamed G., Johns Hopkins Medicine, Baltimore, Maryland, United States

Mohamed G. Atta,

Background

Retroperitoneal fibrosis (RPF) is a rare disease characterized by fibroinflammatory tissue surrounding the abdominal aorta and iliac arteries. Due to the nonspecific nature of its clinical presentation, diagnosis is challenging and often delayed. This study aims to comprehensively analyze the clinical characteristics, treatment approaches, and renal outcomes in a diverse cohort of RPF patients.

Methods

Electronic Health Records of patients who underwent evaluation for RPF between 01/2015 to 09/2022 at Johns Hopkins Hospital were reviewed to collect data on baseline demographics, clinical information, laboratory findings, imaging results, histopathology, and outcomes. Follow-up was from the first nephrology visit until end of study or last follow-up.

Results

43 patients underwent evaluation for RPF. The most common lesion was idiopathic RPF which was present in 23 (54%) patients (mean age: 62 years, 48% Male; 35% Black, mean eGFR: 58 ml/min/1.73m³), followed by Erdheim Chester Disease (ECD) in 4 (9%) patients (mean age: 68 years, 75% Male, 0% Black, mean eGFR: 81 ml/min/1.73m³), and IgG4 disease in 4 (9%) patients (mean age: 58 years, 100% Male, 75% Black, mean eGFR: 58 ml/min/1.73m³). The remaining 7 (16%) patients (mean age: 62 years, 57% Male, 43% Asian, mean eGFR: 74 ml/min/1.73m³) had diverse diagnoses, including Follicular Lymphoma, Secondary RPF (from asbestos and Renal Cell Carcinoma), Non-Langerhans Cell Histiocytosis, Thrombosed Aneurysm, and Adnexal Cyst. 5 (12%) patients are currently under evaluation. Common symptoms included pain (flank, abdominal, or back), emesis, and ≥ 5lb weight loss. Imaging showed hydronephrosis in most patients and periaortic findings in those with IgG4 disease and idiopathic RPF. 19 (44%) patients had acute kidney injury at diagnosis. In the first year, 17 (74 %) idiopathic RPF patients received medical therapy (Mycophenolate, Rituximab, Tamoxifen, or Steroids), and patients who tolerated medical therapy had stable or improved disease outcomes. IgG4 disease and ECD patients received immunosuppressants and ECD-specific treatment after biopsy confirmation resulting in improved or stable disease during follow-up.

Conclusion

This study offers valuable insights on diagnosing, managing, and evaluating patients with RPF and related conditions. Our diverse cohort enhances understanding of this rare disease and informs clinical decision-making in RPF cases.