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IdiopathicRetroperitonealFibrosis Augusto VaglioFederica Maritati2016 год

Idiopathic Retroperitoneal Fibrosis
review

Страница публикацииПубликация в OpenAlex
Аннотация: Idiopathic retroperitoneal fibrosis (RPF), reviewed herein, is a rare fibro-inflammatory disease that develops around the abdominal aorta and the iliac arteries, and spreads into the adjacent retroperitoneum, where it frequently causes ureteral obstruction and renal failure. The clinical phenotype of RPF is complex, because it can be associated with fibro-inflammatory disorders involving other organs, is considered part of the spectrum of IgG4-related disease, and often arises in patients with other autoimmune conditions. Obstructive uropathy is the most common complication, although other types of renal involvement may occur, including stenosis of the renal arteries and veins, renal atrophy, and different types of associated GN. Environmental and genetic factors contribute to disease susceptibility, whereas the immunopathogenesis of RPF is mediated by different immune cell types that eventually promote fibroblast activation. The diagnosis is made on the basis of computed tomography or magnetic resonance imaging, and positron emission tomography is a useful tool in disease staging and follow-up. Treatment of idiopathic RPF aims at relieving ureteral obstruction and inducing disease regression, and includes the use of glucocorticoids, combined or not with other traditional immunosuppressants. However, biologic therapies such as the B cell–depleting agent rituximab are emerging as potentially efficacious agents in difficult-to-treat cases.
Год издания: 2016
Авторы: Augusto Vaglio, Federica Maritati
Издательство: American Society of Nephrology
Источник: Journal of the American Society of Nephrology
Ключевые слова: IgG4-Related and Inflammatory Diseases, Pancreatitis Pathology and Treatment, Amyloidosis: Diagnosis, Treatment, Outcomes
Другие ссылки: Journal of the American Society of Nephrology (HTML)
Europe PMC (PubMed Central) (HTML)
PubMed Central (HTML)
PubMed (HTML)