Idiopathic retroperitoneal fibrosis (RPF) is a rare condition included in the spectrum of IgG4-related disease characterized by a fibroinflammatory tissue usually surrounding the abdominal aorta and iliac arteries. It was initially thought to result from a reaction to aortic atherosclerosis; however, given its frequent association with autoimmune diseases and its sensitivity to immunosuppressive therapies, it is now considered a systemic autoimmune condition. The genetic basis of the disease is poorly investigated. We performed a large-scale immunogenetic study of idiopathic RPF by using the Immunochip, an array focused on autoimmunity-associated gene variants.
A large-scale genetic analysis reveals an autoimmune origin of idiopathic retroperitoneal fibrosis
Emmi G.;
2018-01-01
Abstract
Idiopathic retroperitoneal fibrosis (RPF) is a rare condition included in the spectrum of IgG4-related disease characterized by a fibroinflammatory tissue usually surrounding the abdominal aorta and iliac arteries. It was initially thought to result from a reaction to aortic atherosclerosis; however, given its frequent association with autoimmune diseases and its sensitivity to immunosuppressive therapies, it is now considered a systemic autoimmune condition. The genetic basis of the disease is poorly investigated. We performed a large-scale immunogenetic study of idiopathic RPF by using the Immunochip, an array focused on autoimmunity-associated gene variants.Pubblicazioni consigliate
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