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Chronic Periaortitis: an Update

  • Orphan Diseases (B Manger, Section Editor)
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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.

Summary

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.

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Acknowledgments

We gratefully thank Daniele Venneri for his help in preparing the figures.

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Authors and Affiliations

  1. Nephrology Unit, Parma University Hospital, Parma, Italy

    Alessandra Palmisano, Federica Maritati & Augusto Vaglio

  2. Unità Operativa di Nefrologia, Azienda Ospedaliero-Universitaria di Parma, Via Gramsci 14, 43126, Parma, Italy

    Augusto Vaglio

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  1. Alessandra Palmisano
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  2. Federica Maritati
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Correspondence to Augusto Vaglio.

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Palmisano, A., Maritati, F. & Vaglio, A. Chronic Periaortitis: an Update. Curr Rheumatol Rep 20, 80 (2018). https://doi.org/10.1007/s11926-018-0789-2

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