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Aortic Disease
Published in Paul Schoenhagen, Frank Dong, Cardiac CT Made Easy, 2023
An occasional finding with aneurysms typically of the abdominal aorta is a rim of soft tissue (suspected inflammatory tissue). These aneurysms are described as inflammatory aneurysms (Figure 10.33).150 There is an overlap with retroperitoneal fibrosis.
Unexplained Fever In Urology
Published in Benedict Isaac, Serge Kernbaum, Michael Burke, 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
The oesophagus
Published in Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie, Bailey & Love's Short Practice of Surgery, 2018
Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie
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.
IgG4 related disease and aortitis: an up-to-date review
Published in Scandinavian Journal of Rheumatology, 2023
N Jayachamarajapura Onkaramurthy, SC Suresh, P Theetha Kariyanna, A Jayarangaiah, G Prakash, B Raju
Active and symptomatic patients with IgG4-RD need treatment to decrease inflammation, maintain remission, and preserve organ function (67). Asymptomatic aortitis or periaortitis with or without retroperitoneal fibrosis needs treatment. In the case of retroperitoneal fibrosis causing urinary tract obstruction, hydronephrosis, and post-renal acute kidney injury, relieving the obstruction should be the priority, followed by immunosuppression. Most patients (> 90%) respond to a moderate dose (0.5 mg/kg) of steroids (17). It is crucial to be cautious with steroids in large aneurysms as they are prone to rupture. Ozawa et al documented that 20% of the patients showed aortic luminal dilatation post-steroid therapy, although they responded well to steroids (68). Hence, it would be wise to weigh the benefits versus risks when considering steroid therapy in patients with aneurysmal dilatation. But there are no clear guidelines or data on dose or on the size of aortic aneurysms that are at increased risk of rupture post-steroid therapy. There is anecdotal evidence of aneurysmal rupture post-steroid therapy (69). A small proportion of patients (n = 2) with aortic luminal dilatation showed an increase in luminal dilatation after treatment with glucocorticoids (70). The recent large prospective study of IgG4-related aortic disease did not report the incidence of rupture or significant increase in aneurysmal size following moderate-dose steroids in patients with IgG4-related aortic disease (17).
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
IgG4-related periaortitis/periarteritis: An under-recognized condition that is potentially life-threatening
Published in Modern Rheumatology, 2019
Ichiro Mizushima, Satomi Kasashima, Yasunari Fujinaga, Mitsuhiro Kawano, Nobukazu Ishizaka
In China, Lin et al. reported the results of a prospective study investigating the clinical characteristics of IgG4-related disease, in which they identified 31 (26%) patients with periaortitis/retroperitoneal fibrosis among 118 patients diagnosed with IgG4-related disease [42]. Lin et al. utilized CDC for the diagnosis of IgG4-related disease, but tissue analysis for 68 (58%), and made a definitive diagnosis for 64 (54%). In addition, Chen et al. assessed 28 patients who were diagnosed with definitive IgG4-related disease by CDC; thus, histological assessment was performed for all patients enrolled [43]. They identified 1 (4%) case of aortic involvement and 3 (11%) cases of retroperitoneal fibrosis; however, it was not reported whether tissue sampling of these tissues was carried out.