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 / Martorana D., Marquez A., Carmona F.D., Bonatti F., Adorni A., Urban M.L., Maritati F., Accorsi Buttini E., Marvisi C., Palmisano A., Rossi G.M., Trivioli G., Fenaroli P., Manenti L., Nicastro M., Incerti M., Gianfreda D., Bani S., Ferretti S., Corradi D., et al.. - In: JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY. - ISSN 0091-6749. - ELETTRONICO. - 142:(2018), pp. 1662-1665. [10.1016/j.jaci.2018.06.045]
A large-scale genetic analysis reveals an autoimmune origin of idiopathic retroperitoneal fibrosis
Adorni A.;Urban M. L.;Trivioli G.;Nicastro M.;Incerti M.;Corradi D.;Emmi G.;Di Scala G.;Martin J.;Vaglio A.
2018
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.
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