Idiopathic retroperitoneal fibrosis: an update for nephrologists

Abstract

Idiopathic retroperitoneal fibrosis (IRF) is a rare condition characterized by the development of a peri-aortic and peri-iliac tissue showing chronic inflammatory infiltrates and pronounced fibrosis. Ureteral entrapment with consequent obstructive uropathy is one of the most common complications of IRF, which can lead to acute renal failure and, in the long term, to varying degrees of chronic kidney disease. IRF may be isolated or develop in association with autoimmune diseases (e.g. Hashimoto’s thyroiditis and psoriasis) and other fibro-inflammatory disorders (often within the spectrum of immunoglobulin G4-related disease), which suggests that it should be considered as a potentially systemic condition. IRF is an immune-mediated disease: genetic variants (e.g. human leukocyte antigen (HLA)-DRB1*03) and environmental agents (mainly exposure to asbestos and smoking) are strongly associated with an increased risk of developing the disease, while a complex network of chemokines (e.g. CXCL12 and C-C moti chemokine 11 (CCL11)) and cytokines [e.g. interleukin (IL)-6, IL-12 and IL-13] is likely to orchestrate the inflammatory response and simultaneously promote fibrosis. Glucocorticoids, alone or in combination with traditional immunosuppressants such as methotrexate and mycophenolate mofetil, are usually efficacious and promptly induce disease remission; however, up to 50% of patients relapse, thus requiring repeat immunosuppressive courses. Biologic drugs, namely rituximab, are being explored for the treatment of IRF. In addition to medical therapies, interventional procedures (mainly ureteral stenting) are required to relieve ureteral obstruction, whereas surgical ureterolysis is generally reserved to refractory cases. If appropriately treated, then the overall and renal prognosis of IRF are good, with <5% patients developing end-stage renal disease.

Renal Involvement in Retroperitoneal Fibrosis: Prevalence, Impact and Management Challenges

“…Some typical computed tomography (CT) and/or magnetic resonance imaging (MRI) findings of IRPF have been described 15 although they are not disease-specific, and a comprehensive approach considering clinical and radiological correlations is still needed. Compared with the findings in secondary RPF (eg malignancy), the typical CT/MRI findings of IRPF include soft tissue masses surrounding anterolaterally a infrarenal abdominal aorta (as opposed to malignant RPF which usually extends to a suprarenal aorta) and/or proximal common iliac arteries without displacing them, a tendency for the ureters to deviate medially (as opposed to lateral displacement by malignant RPF), and absence of infiltration into muscle or bone ( Figure 1 ).…”

“…Such renal involvement in RPF can lead to not only acute kidney injury (AKI) but also chronic kidney disease (CKD) including renal atrophy or even end-stage renal disease (ESRD). 15 Accordingly, the epidemiology, pathophysiology, clinical features, and management of RPF need to be clarified.…”

Generalized pustular psoriasis complicated with idiopathic retroperitoneal fibrosis successfully treated with infliximab

“…Idiopathic retroperitoneal fibrosis (IRF) is a rare disease characterized by the development of peri-aortic and peri-iliac tissues showing chronic inflammatory infiltrate and pronounced fibrosis. 1 To the best of our knowledge, this is the first report of GPP complicated with IRF successfully treated with the anti-tumor necrosis factor (TNF)α antibody infliximab.…”

“…with autoimmune disease including psoriasis. 1 Interleukin-17 could present the link between T-cell activation and psoriasis and the trigger of the fibrosing periaortitis ultimately leading to IRF. 2 A recent study on 113 IRF patients found that 21% of them had high IgG4 levels at diagnosis.…”

Renal Involvement in IgG4-Related Disease: From Sunlight to Twilight

“…IgG4-RD RF benefits of the same treatments already described. Notably, recent reports of MMF and RTX treatment in RF show efficacy whether or not it is a manifestation of IgG4-RD (60,61). Some conditions, such as smoking habit, AKI at diagnosis, ANA positivity and lumbar pain, are associated to relapse, and all these factors represent a negative prognostic combination for renal outcome (62).…”