The oesophagus
Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie in Bailey & Love's Short Practice of Surgery, 2018
This rare condition can occur alone or together with retroperitoneal fibrosis. The cause is unknown and, although the major consequences are usually cardiovascular as a result of caval compression, dysphagia can occur. The existence of irreparable cardiovascular problems usually precludes surgical intervention on the oesophagus.
Unexplained Fever In Urology
Benedict Isaac, Serge Kernbaum, Michael Burke in Unexplained Fever, 2019
The process may be idiopathic or secondary. Causes of secondary retroperitoneal fibrosis include malignancy, injury, infection, drugs and auto-immune process such as vasculitis.130-132 When these are excluded, it may be considered idiopathic.128,129
64 MS-CTU: Review of techniques and spectrum of the ureteric diseases
Published in Alexandria Journal of Medicine, 2018
Primary retroperitoneal fibrosis (PRPF) was diagnosed in only one patient (0.025%), during the whole study (Fig. 13). It is a rare disease with an estimated incidence of 1.38/100,000 people. Some medications, abdominal aortic aneurysm, infections, malignancies, trauma, surgery, and radiation therapy have been accused to be causative pathology of RPF; however, it is commonly idiopathic. PRPF commonly occurs in individuals 40–60 years of age; with a male to female ratio of 3.3:1.30,31 It had been previously described as chronic periaortitis, as the pathological process is centered around the abdominal aorta. Histologically; it is a fibrotic reaction within the retroperitoneal tissues that encases the retroperitoneal structure causing bilateral medial ureteral traction and constriction. IVP can show the secondary effect of PRPF, which is bilateral medial ureteric deviation and obstructive uropathy. However, CTU will be more informative imaging tool, as it can clearly show the extent of the paraortic retroperitoneal fibrotic bands with its traction obstructive effect on the ureters. Also, it helps to show if there is a causative abdominal etiology like abdominal aortic aneurysm or retroperitoneal malignancy which are the common causes of secondary RPF.32
Successful treatment of sclerosing mesenteritis with tamoxifen monotherapy
Published in Baylor University Medical Center Proceedings, 2023
Lauren Zammerilla Westcott, Dallas Wolford, Taylor G. Maloney, Ronald C. Jones
Given the lack of therapeutic trials, treatment recommendations are based on case reports, small case series, and trials in other fibrosing diseases such as idiopathic retroperitoneal fibrosis.2 Tamoxifen has been described as a treatment option for retroperitoneal fibrosis, specifically for patients who cannot tolerate high-dose glucocorticoids.7 The mechanism by which tamoxifen exerts its effect is unknown but is thought to be hormonal independent. It has been suggested that tamoxifen affects growth factors that inhibit fibroblast proliferation, which may explain its role in the treatment of both retroperitoneal fibrosis and sclerosing mesenteritis. Its antiangiogenic and antiestrogen properties may also contribute to its efficacy, perhaps suppressing inflammation and immune-mediated responses.7
Idiopathic retroperitoneal fibrosis: a cross-sectional study of 142 Chinese patients
Published in Scandinavian Journal of Rheumatology, 2018
S Liao, Y Wang, K Li, J Zhu, J Zhang, F Huang
Idiopathic retroperitoneal fibrosis (iRPF) is considered as a rare disease. In this chronic inflammatory disorder, the tissues are infiltrated by lymphoplasmacytic cells and lateral deposits of thickened fibrosis, with the fibrous mass enveloping the abdominal aorta and iliac vessels as well as nearby structures or organs, resulting in flank pain, lower extremity oedema, hydronephrosis, and other clinical symptoms (1). iRPF affects 0.1–1.3 cases/million people per year (2). Diagnostic criteria do not exist and, therefore, diagnosis is subject to clinical opinion, resulting in cases with a wide range of symptoms and definitions. Before the 1990s, the disease was regarded as a consequence of local inflammatory reactions to atherosclerotic plaques (3, 4). However, the presence of constitutional symptoms, high concentrations of acute-phase reactants, and accompanying autoimmune disease suggests that it may be a systemic autoimmune or inflammatory disease (1). Currently, iRPF is considered to come under the immunoglobulin G4 (IgG4)-related disease umbrella (5). Glucocorticoids are highly effective in treating the constitutional symptoms of iRPF (6–9); nevertheless, combined therapy using immunosuppressants with glucocorticoids is normally used.
Related Knowledge Centers
- Aorta
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- Immunosuppression
- Kidney Failure
- Low Back Pain
- Hypertension
- Autoimmune Disease
- Urinary System
- Retroperitoneal Space
- Kidney