Retroperitoneal fibrosis: retrospective descriptive study on clinical features and management

Abstract

IntroductionRetroperitoneal fibrosis (RPF) is a rare condition characterized by the presence of inflammatory and fibrous retroperitoneal tissue that often encases the ureters or abdominal organs. This study describes the clinical characteristics, diagnostic methods, and treatments and their effects on renal function.MethodsWe conducted a retrospective analysis of patients diagnosed with RPF at Maisonneuve-Rosemont Hospital.ResultsWe identified 17 patients with RPF between 1998 and 2013. Eight patients were females (47%), and the mean age was 62±18 years. Eleven patients were idiopathic. Back pain was the most common symptom. All diagnoses were made based on the finding of a retroperitoneal mass on the computed tomography scan. Three patients had histological diagnosis of RPF and seven patients had unspecific changes on their biopsy. Twelve patients needed double-J stents, three patients had a temporary percutaneous nephrostomy, two patients had to have a nephrectomy for refractory ureteral obstruction, and one patient required hemodialysis. Ten patients with idiopathic RPF received medical treatment. In the treated group, only two patients had complete remission of the disease and five patients had improvement of their lesions. There were no deteriorations and only one relapse. Seven patients did not receive any treatment; two of them achieved complete remission, one of them deteriorated, and two of them had no changes.ConclusionMost of our cases of RPF were idiopathic. Almost all treated patients received prednisone and seemed to respond, at least partially. There was a lot of heterogeneity in patient management, which makes it difficult to compare treatment effects. However, treated patients seemed to have more favorable outcomes than those who were not.

Renal fungus ball in a patient with retroperitoneal fibrosis: Unique complication in a rare disease

“…This disease is characterised by chronic inflammation of the retroperitoneum, which leads to entrapment and obstruction of the peritoneal structures including the ureters . Consistent with the Review published studies, our patient also had ureteric obstruction which predisposed him to have renal fungus ball. Despite appropriate therapy, the patient experienced recurrences of fungus ball.…”

Idiopathic retroperitoneal fibrosis causing unilateral ureteral and sigmoid colon obstruction

“…[ 1 , 2 ] Its etiology and pathogenesis are unclear. [ 2 – 4 ] It can be classified as idiopathic and secondary. [ 2 , 5 ] It was first described in 1905 by the French urologist Albarran and became fully described as an entity in 1948 by Ormond.…”

“…[ 3 ] Laboratory finding may include elevated erythrocyte sedimentation rate, elevated C-reactive protein, elevated urea and creatinine levels, anemia, polyclonal hypergammaglobulinemia, elevated alkaline phosphatase, and presence of antinuclear antibodies. [ 4 , 7 , 8 ] Urine examination is usually normal, but may reveal microscopic hematuria or pyuria. [ 4 , 7 , 8 ] Ultrasound usually reveals extensive, well-defined, hypoechoic mass accompanied with various degree of hydronephrosis.…”

Retroperitoneal fibrosis associated with orbital pseudotumor without evidence of IgG4: A case report with review of literature

“…Systemic symptoms are frequent and include malaise, fatigue, nausea, vomiting, anorexia, myalgia and loss of weight. Lower extremity edema, deep vein thrombosis, mesenteric ischemia, claudication of upper and lower extremities, scrotal swelling, varicocele, hydrocoele or secondary arterial hypertension are complications that appear as a result of the compression of lymphatic and blood vessels (2,3,11). Clinical features and laboratory findings are nonspecific, therefore IRF is a diagnosis of exclusion (3).…”