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Gastrointestinal aspects of vasculitides

Nature Reviews Gastroenterology & Hepatology volume 14, pages 185–194 (2017)Cite this article

Subjects

Key Points

  • Gastrointestinal manifestations are rarely the predominating features of systemic vasculitides but can rapidly become life-threatening

  • Gastrointestinal involvement is rare in large-vessel vasculitides and is mainly due to large-vessel stenosis or occlusion, with long-segment gastrointestinal tract ischaemic manifestations

  • Small-vessel vasculitides can cause various gastrointestinal manifestations, including mucosal purpura (risk of haemorrhage), patchy granulomatous or ischaemic ulcerations that can mimic IBD and can perforate

  • Liver involvement is rarely clinically significant in systemic vasculitides, except for rare infarcts due to large or medium-sized vasculitic occlusions and Budd–Chiari syndrome in patients with Behçet's disease

  • Treatment of gastrointestinal manifestations must be prompt, adapted to the severity of the disease and conducted in collaboration with general surgery and interventional radiology as needed

  • Patients with single-organ gastrointestinal vasculitis must be followed closely for the development of systemic vasculitis, although such a progression is observed in a maximum of one-quarter of patients over the subsequent 5 years

Abstract

Systemic vasculitides are caused by inflammation of blood vessels and can affect any organ and any part of the gastrointestinal tract, hepatic and biliary system, as well as the pancreas. These disorders can cause a wide array of gastrointestinal manifestations, from asymptomatic elevated transaminase levels and mild abdominal pain to potentially life-threatening bowel perforations and peritonitis. A diagnosis based solely on gastrointestinal symptoms is challenging as these manifestations are not specific. Conversely, diagnostic and therapeutic delays can be rapidly detrimental. In this article, we review the epidemiology, characteristics and management of the main gastrointestinal manifestations of systemic vasculitides, including polyarteritis nodosa and antineutrophil cytoplasm antibody-associated vasculitides, as well as isolated vasculitides limited to the gastrointestinal tract.

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Figure 1: Chronic ischaemic colitis in a female patient with Takayasu arteritis.
Figure 2: Jejunitis in a female patient with IgA vasculitis.

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Acknowledgements

M.S. is supported by a Vasculitis Clinical Research Consortium Fellowship grant (2015–2016). The Vasculitis Clinical Research Consortium has received support from the National Institute of Arthritis and Musculoskeletal and Skin Diseases (U54AR057319 and U01AR5187404), the National Center for Research Resources (U54RR019497), the Office of Rare Diseases Research, and the National Center for Advancing Translational Science.

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Authors and Affiliations

  1. Division of Rheumatology, Vasculitis Clinic, Mount Sinai Hospital, University of Toronto, 60 Murray Street, Toronto, M5T 3L9, Ontario, Canada

    Medha Soowamber & Christian Pagnoux

  2. Division of Gastroenterology, Mount Sinai Hospital, University Health Network, University of Toronto, 437–600 University Avenue, Toronto, M5G 1X5, Ontario, Canada

    Adam V. Weizman

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  1. Medha Soowamber
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C.P. and M.S. contributed equally to researching data for the article, contributing to discussion of content and writing. All authors contributed equally to reviewing and/or editing the manuscript before submission.

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Correspondence to Christian Pagnoux.

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Competing interests

C.P. reports receiving fees for serving on advisory boards from Chemocentryx, GlaxoSmithKline and Hoffman-LaRoche; he also reports lecture fees and grant support from Bristol-Myers Squibb, EuroImmune, Hoffman-LaRoche and Terumo-BCT.

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Soowamber, M., Weizman, A. & Pagnoux, C. Gastrointestinal aspects of vasculitides. Nat Rev Gastroenterol Hepatol 14, 185–194 (2017). https://doi.org/10.1038/nrgastro.2016.179

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