Vasculitides of the gastrointestinal tract

ArticleinSeminars in Diagnostic Pathology 26(2):77-88 · May 2009with13 Reads
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.
    • "Prognosis of untreated patients has been improved by use of steroids with 48% survival at 5 years. The combination of cyclophosphamide with prednisolone results in 96% remission (Ahn et al., 2009). "
    [Show abstract] [Hide abstract] ABSTRACT: Polyarteritis nodosa is a form of vasculitis that affects several organs. Gastrointestinal involvement is frequent, but cases in which the gastrointestinal tract is the only site of disease are rare. In this paper, we report a case of a 40 -year- old patient with polyarteritis nodosa restricted to small part of the small intestine, which underwent resection and anastomosis. Laparotomy was performed despite normal radiological findings. He underwent resection of the infracted segment. Histopathological examination confirmed the diagnosis. Thus, a pathological diagnosis of ischaemic infarction due to polyarteritis Nodosa (PAN) was made. The patient had no other signs of chronic vasculitis and for this reason surgical treatment resolved the clinical symptoms.
    Full-text · Article · Mar 2015
    • "Previous reports of CSS provided only a few details regarding small intestinal ulcerations. Double-balloon endoscopy (DBE) is a relatively new endoscopic device designed to visualize the entire small intestine [5] . Observation of the entire small intestine can be performed using an oral and/or anal DBE approach. "
    [Show abstract] [Hide abstract] ABSTRACT: Churg-Strauss syndrome (CSS) is a systemic vascular disorder characterized by severe bronchial asthma, hypereosinophilia, and allergic rhinitis. Small intestinal ulcers associated with CSS are a relatively rare manifestation that causes gastrointestinal bleeding. Multiple deep ulcers with an irregular shape are characteristic of small intestinal involvement of CSS. Video-capsule-endoscopy (VCE), double-balloon endoscopy (DBE) and Spirus assisted enteroscopy have been developed recently and enabled observation of the small intestine. In this case report, we have described a patient with CSS who had multiple deep ulcers in the jejunum detected by oral DBE. Since severe gastrointestinal (GI) involvement has been identified as an independent factor associated with poor outcome, the careful investigation of GI tract must be needed for CSS patients with GI symptoms. We describe the usefulness of DBE for diagnosis of small intestinal ulcers in patient with CSS.
    Full-text · Article · May 2012
    • "2.3.1 Acute Manifestations of Vasculitis on the Small Intestine (Ha et al. 2000; Ahn et al. 2009) The majority of forms of vasculitis, in any case those concerning small-and medium-sized vessels, may affect the small intestine. We should think of them each time; we see images in a young patient suggesting ischaemic lesions in an unusual site, for instance in the stomach, above all the duodenum (ischaemia in the duodenum is practically always related to vasculitis), or the rectum, or when the small intestine and colon are simultaneously diffusely affected or when other viscera are affected, e.g. the uro-genital system. "
    [Show abstract] [Hide abstract] ABSTRACT: In acute gastric or small bowel conditions, whether they are infectious, inflammatory, or ischemic, the CT scan objectifies submucosal oedema with parietal stratification, producing ‘target’ or ‘double-halo’ images that can be easily analysed in venous time. Precise analysis of proximal peritoneal reactions and of endoluminal content are the first steps of the diagnostic approach. In an acute clinical context, a number of hypotheses must be discussed (perforation, infection, arterial ischaemia, capillary hyperpermeability, congestion by portal venous stasis, etc.), some of which may be supported by abdominal/pelvic exploration, as a general rule complemented by thoracic exploration if there are no contraindications for the radiation risk (young subjects and women of childbearing potential). In all cases, the clinical context and laboratory tests are fundamental for orientating the diagnosis: a history of abdominal pain and diarrhoea, a state of acquired immunosuppression, a recent stay in a country where there are endemic parasites, a purpuric rash on the lower limbs, a marked inflammatory syndrome seen in laboratory tests, etc. are all signs providing pointers for the right direction which one needs to know how to find out by precise, directed questioning and clinical examination
    Full-text · Chapter · Sep 2011
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