Alessandra Palmisano1, Federica Maritati1, Augusto Vaglio2,3. 1. Nephrology Unit, Parma University Hospital, Parma, Italy. 2. Nephrology Unit, Parma University Hospital, Parma, Italy. augusto.vaglio@virgilio.it. 3. Unità Operativa di Nefrologia, Azienda Ospedaliero-Universitaria di Parma, Via Gramsci 14, 43126, Parma, Italy. augusto.vaglio@virgilio.it.
Abstract
PURPOSE OF REVIEW: We aim to review traditional concepts and recent developments on the nosology, pathophysiology, clinical phenotypes and treatment of chronic periaortitis (CP). RECENT FINDINGS: CP is a rare disorder hallmarked by a periaortic fibro-inflammatory tissue. It can present as an isolated disease, but it can also be associated with other autoimmune and fibro-inflammatory lesions (e.g., fibrosing mediastinitis, sclerosing pancreato-cholangitis) that are part of the spectrum of IgG4-related disease. In a subgroup of patients, it also involves the thoracic aorta (so-called "diffuse periaortitis"), which supports the notion of an inflammatory disorder of large arteries. The pathogenesis of CP is multifactorial: recent studies have elucidated the predisposing role of immunogenetic variants and exposures to environmental agents such as smoking and asbestos. CP is a rare immune-mediated disease that affects the abdominal aorta and the iliac arteries and, in some cases, the thoracic aorta. It may overlap with manifestations of IgG4-related disease, and its treatment comprises glucocorticoids, conventional and biological immunosuppressive agents.
PURPOSE OF REVIEW: We aim to review traditional concepts and recent developments on the nosology, pathophysiology, clinical phenotypes and treatment of chronic periaortitis (CP). RECENT FINDINGS: CP is a rare disorder hallmarked by a periaortic fibro-inflammatory tissue. It can present as an isolated disease, but it can also be associated with other autoimmune and fibro-inflammatory lesions (e.g., fibrosing mediastinitis, sclerosing pancreato-cholangitis) that are part of the spectrum of IgG4-related disease. In a subgroup of patients, it also involves the thoracic aorta (so-called "diffuse periaortitis"), which supports the notion of an inflammatory disorder of large arteries. The pathogenesis of CP is multifactorial: recent studies have elucidated the predisposing role of immunogenetic variants and exposures to environmental agents such as smoking and asbestos. CP is a rare immune-mediated disease that affects the abdominal aorta and the iliac arteries and, in some cases, the thoracic aorta. It may overlap with manifestations of IgG4-related disease, and its treatment comprises glucocorticoids, conventional and biological immunosuppressive agents.
Authors: Davide Martorana; Augusto Vaglio; Paolo Greco; Adele Zanetti; Gabriella Moroni; Carlo Salvarani; Mario Savi; Carlo Buzio; Tauro M Neri Journal: Arthritis Rheum Date: 2006-02-15
Authors: Arezou Khosroshahi; Mollie N Carruthers; John H Stone; Shweta Shinagare; Nisha Sainani; Robert P Hasserjian; Vikram Deshpande Journal: Medicine (Baltimore) Date: 2013-03 Impact factor: 1.889