Vasculitides of the gastrointestinal tract. Semin Diag Pathol

Department of Pathology, Toronto General Hospital/University Health Network, Toronto, ON, Canada.
Seminars in Diagnostic Pathology (Impact Factor: 2.56). 05/2009; 26(2):77-88. DOI: 10.1053/j.semdp.2009.06.001
Source: PubMed


Systemic vasculitis is often not considered as a possible diagnosis by clinicians because of its low prevalence compared with other more common diseases. Vasculitis can affect any end organ, and it is therefore often missed early on in disease progression. Gastrointestinal (GI) manifestations of vasculitis are considered rare and the presentation is often nonspecific. However, if there is significant involvement of the major vessels of the gastrointestinal system, life-threatening sequelae, including perforation and bowel ischemia, may occur. This makes early and immediate management crucial to improve long-term morbidity and mortality. Diagnosis of various GI vasculitides often relies on correlation of clinical manifestations with pathology and additional investigations. This paper reviews the various vasculitides that affect the GI tract, including systemic lupus erythematosus, mixed connective tissue disease, Henoch Schönlein purpura, polyarteritis nodosa, Churg-Strauss syndrome, Wegener's granulomatosis, microscopic polyangiitis, enterocolic lymphocytic phlebitis, and Behcet's disease. Segmental arterial mediolysis, mistakenly believed to be a vasculitis, is also discussed.

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    ABSTRACT: Les vascularites systémiques peuvent être primitives, classées selon le calibre des vaisseaux atteints, ou secondaires, conséquence d’une infection, d’une prise médicamenteuse, d’un cancer ou d’une autre maladie systémique comme la polyarthrite rhumatoïde. Des manifestations digestives peuvent survenir, à des fréquences variables, au cours de ces vascularites et révèlent la maladie chez environ 15 % des patients. Les douleurs abdominales, les nausées, les vomissements ou les diarrhées sont fréquents, mais peu spécifiques. Une inflammation, un infiltrat purpurique et/ou nécrotique et des ulcérations (parfois d’allure granulomateuse) des muqueuses sont possibles. Les atteintes ischémiques, les infarctus et les perforations intestinales, surtout de l’intestin grêle, sont plus graves, de même que les atteintes pancréatiques et les hémorragies digestives (notamment par rupture de microanévrismes). La prise en charge combinée médicale, et chirurgicale si nécessaire, doit être précoce. La survie à dix ans des patients atteints de vascularite systémique nécrosante primitive est, aujourd’hui, de l’ordre de 80 %, en l’absence d’atteinte digestive ou si les manifestations digestives sont mineures. Le taux de survie à cinq ans n’est, par contre, que de 56 % en cas de manifestation digestive grave, chirurgicale. Primary systemic vasculitides are rare and primarily classified according to the size of the vessels predominantly affected. Secondary vasculitides can be caused by infection or drug or develop as a complication of cancer or other systemic disease like rheumatoid arthritis. Gastrointestinal manifestations occur at variable frequencies in vasculitis patients and may reveal the disease (15% of the patients). Abdominal pain, nausea, vomiting, and diarrheas are common, but non specific, manifestations. Mucosal inflammation, purpuric infiltrate or necrosis and ulceration (possibly with a granulomatous appearance) can also be observed. Gastrointestinal ischemia, bowel infarction, intestinal perforation, especially of the small bowel, pancreatitis, and gastrointestinal bleeding are more severe manifestations. Prompt and combined medical and, when necessary, surgical management is mandatory. Ten-year survival rate of patients with primary systemic necrotizing vasculitides is now about 80% in the absence of severe visceral involvement or in case of only minor gastrointestinal manifestations. Conversely, at 5 years, survival rate is only 56% for patients with severe and surgical gastrointestinal manifestations. Mots clésVascularite-Perforation-Ischémie mésentérique-Vaisseau KeywordsVasculitis-Perforation-Mesenteric ischemia-Vessel
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