E001 Retroperitoneal fibrosis patients need a dedicated multidisciplinary team

Abstract

Abstract Background/Aims Retroperitoneal fibrosis is a rare chronic inflammatory fibrosing condition of the retroperitoneal tissues. Inflammation can cause tethering and occlusion of nearby structures, especially the ureters, leading to acute obstruction as well as long-term renal impairment. This condition usually either presents with nonspecific abdominal pain or when the patient is in crisis due to urological obstruction. There is little known about its treatment beyond steroids. A significant proportion of patients are at risk of relapse. We characterised patients with retroperitoneal fibrosis in our heath board to better understand how these patients present and what the unmet needs in their management are. Methods Patients were identified by contacting rheumatologists and by searching the urological stent registry from 2019 to 2022. The records were searched for demographics, events around diagnosis, initial treatment, investigations, including imaging reports and pathology, as well as follow up clinic letters detailing any relapses and complications. Results We found 22 patients in the health board with idiopathic retroperitoneal fibrosis. Half had sole care under urology. Only 6 received joint care from urology and rheumatology. Demographics of these patients were comparable to literature (mean age 60 (range 43-81 years), female to male ratio (1:2.6), compared to the reported mean age of 55-60 and female to male ratio of 1:2). Diagnosis was usually made by CT scan. The mean incidence of new diagnosis was 2 per year over the last 10 years in a catchment population of 850,000. In 19 patients with treatment information available, 4 never received steroids or immunomodulation therapy. For those that received steroid therapy, doses ranged from 0.2mg/kg to 0.8mg/kg over a range of 1 week to 6 months of maximum dose duration. 73% of patients now have chronic renal dysfunction with eGFR <60ml/min/1.73m2, and 27% with stage 4 renal failure with eGFR <30 ml/min/1.73m2. One of these patients had end stage renal disease and is deceased. Conclusion This characterisation study has shown that patients have had heterogenous journeys with their RPF diagnosis and treatment. 73% of these patients have developed chronic renal dysfunction which is dramatically higher than that of the published literature figure of 32%. In order to improve outcomes, there is a need for a standardized approach to diagnosis, treatment and follow up, involving a dedicated multidisciplinary team. Disclosure E. Qian: None. H. Harris: None.