Retroperitoneal Fibrosis in 27 Korean Patients: Single Center Experience

Abstract

Retroperitoneal fibrosis (RPF) is a rare disease with unclear etiology, which is characterized by chronic non-specific inflammation of the retroperitoneum. This study was performed to investigate the clinical characteristics, laboratory findings, radiologic findings, treatment and outcome in Korean patients with RPF. We retrospectively reviewed medical records of 27 RPF patients who were admitted to Yonsei University Medical Center between 1998 and 2009. Twenty-two patients (81%) were male. The mean age at diagnosis was 56 yr. Nine patients had identifiable risk factors of RPF and three patients had combined autoimmune diseases. Acute phase reactants were elevated in most patients. Rheumatoid factor was positive in 3 of 16 patients (19%) and antinuclear antibody in 4 of 17 (24%). Five of 6 patients who were taken positron-emission tomography showed positive uptake. Glucocorticoids were used in 16 patients (59%) and four of them received combination therapy with azathioprine. After immunosuppressive treatment, the levels of acute phase reactants dropped, and the size of mass also decreased in most patients. In conclusion, the clinical characteristics of RPF in Korean patients are similar with other series except for higher proportion of male. Some patients with RPF have autoimmune features. The effect of immunosuppressive treatment on RPF is good.

Retroperitoneal fibrosis: retrospective descriptive study on clinical features and management

“…All our patients had some degree of acute renal failure, and 60.7% remained with some degree of renal failure once treated, which is higher than that in previous studies 1,2,8,9. However, the creatinine was still generally lower at the end of the follow-up period with 41.2% of our patients improving their renal function.…”

“…Diagnosis can be difficult to make, because RPF frequently presents with unspecific symptoms, such as fatigue, back pain, anorexia, weight loss, and with or without urinary symptoms 1,2,8,9. Most of the time, it is found in the setting of acute renal failure investigations.…”

“…Hydroureters and hydronephrosis are frequent. Histopathologic findings are often nonspecific but reflect chronic inflammation and occasionally feature vasculitic findings 1,2,9,14…”

A rare cause of acute post renal failure: Retroperitoneal fibrosis

“…Diagnosis of idiopathic RF is generally delayed due to atypical symptoms of the disease and patients may be diagnosed with postrenal ARF in ED. Urologically, the most frequent and most serious complication of the disease is ARF due to bilateral ureteral obstruction (8,9). In two large studies in the literature, pain was the most common presenting symptom, and additionally undefined symptoms such as fatigue, weakness, myalgia were observed (4, 10).…”

Fibrose rétropéritonéale : revue de littérature

“…Certains auteurs considèrent ces conditions (notamment la chirurgie, la radiothérapie et les médicaments) comme des facteurs prédisposants. 4 La FPR secondaire peut être le résultat d'un anévrisme de l'aorte, d'une radiothérapie antérieure, d'une chirurgie abdominale, de traumatismes, de tumeurs rétropéritoné-ales primaires ou métastatiques, d'infections (tuberculose, histoplasmose, actinomycose) et de la prise de médica-ments (ergotamine, méthyldopa, bêta-bloquants, méthyser-gide, bromocriptine, hydralazine) (tableau 1). 5 Les tumeurs rétropéritonéales primaires, tels que le lymphome ou les sarcomes et les tumeurs métastatiques, tels que le cancer du sein et du côlon, peuvent aussi produire une réaction fibro-inflammatoire et ainsi imiter la FRP.…”