Giant cell (temporal) arteritis: Giant cell (temporal) arteritis is a form of granulomatous arteritis of the aorta and its major branches, with a predilection for the extracranial branches of the carotid artery[1]. It is often associated with polymyalgia rheumatica. The frequency of its GI involvement is rare[5,8].

Takayasu’s arteritis: Takayasu’s arteritis (TA) is a form of granulomatous inflammation of the aorta and its major branches[1].It is characterized by ocular disturbances and decreased brachial artery pulse (pulseless disease). The descending aortic syndrome may cause mesenteric vasculitis, but the frequency of mesenteric or celiac involvement is rare[3,5,6,8]. Although the precise etiology is unknown, the coexistence of TA and ulcerative colitis or Crohn’s disease has been increasingly reported[9,10].

Polyarteritis nodosa: Polyarteritis nodosa (PN) is a form of necrotizing inflammation of medium-sized or small arteries without glomerulonephritis or vasculitis in arterioles, capillaries, or venules[1]. Approximately two-thirds of the patientshave abdominal pain, nausea, vomiting, or other manifestations associated with GI ischemia and infarction[3,5-7]. The clinical course is often dramatic. The typical radiographic feature is an angiographic finding of aneurysms up to 1 cm in diameter within the renal, mesenteric, and hepatic vasculature[3].

Kawasaki’s disease: Kawasaki’s disease is a form of arteritis involving large, medium-sized, and small arteries and is associated with mucocutaneous lymph node syndrome[1]. It usually occurs in children and coronary arteries are often involved. GI involvement is relatively uncommon but acute abdomen with paralytic ileus, ischemic enteritis, and vasculitic appendicitis may occur[6].

Wegener’s granulomatosis: Wegener’s granulomatosis (WG) is a form of granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small-to-medium-sized vessels[1]. GI involvement is relatively rare and granulomatous colitis or gastritis may occur[5].

Churg-Strauss syndrome: Churg-Strauss syndrome(CSS) is a form of eosinophil-rich and granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small-to-medium-sized vessels and is associated with asthma and eosinophilia[1]. GI symptoms of CSS are abdominal pain and diarrhea caused by eosinophilic gastroenteritis (Figure 1)[11].Mesenteric vasculitis may occur, leading to GI ulceration, ischemia, and perforation. Among antineutrophil cytoplasmic antibodies-associated vasculitiswhich include WG, CSS, and microscopic polyangiitis (MPA), GI involvement increases the risk of relapse in CSS[12].

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Figure 1 Churg-Strauss syndrome in a 60-year-old man with fever, abdominal pain, diarrhea, facial swelling, and purpura of the lower extremities. A: Purpura of the right foot; B: Biopsy of the purpura revealed small vessel vasculitis with marked inflammatory infiltrate of eosinophils; C: Colonoscopy disclosed numerous areas of patchy mucosal erythema from the sigmoid colon to the splenic flexure; D: Biopsy of erythema showed mild infiltration of eosinophils around crypts. All figures and legends are reproduced from Hokama et al[11] with permission from Elsevier.

Microscopic polyangiitis: MPA is a form of necrotizing vasculitis with few or no immune deposits affecting small vessels[1]. Although necrotizing glomerulonephritis and pulmonary capillaritis are very common, GI involvement is rare[6].

Henoch-Schönlein purpura: Henoch-Schönlein purpura (HSP) is a form of vasculitis with IgA-dominant immune deposits affecting small vessels[1]. Although HSP is typically a disease of children, adult cases present more severe disease compared to children. It involves the skin, joints, GI tract and kidneys. GI symptoms include colicky abdominal pain and bleeding caused by bowel ischemia and edema. Serious complications include intussusception, infarction, and perforation[6,13]. The descending duodenum and the terminal ileum are frequently involved, with endoscopic characteristics of diffuse mucosal redness, petechiae, hemorrhagic erosions and ulcers[14].Longitudinal ulcers may be clear evidence of mesenteric vascular involvement (Figure 2)[15]. The CT features are bowel wall thickening with the target sign and engorgement of mesenteric vessels with comb sign (Figure 2)[15].

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Figure 2 Henoch-Schönlein purpura in a 38-year-old man with hematochezia. A: Palpable purpura of the right foot; B: Contrast-enhanced computed tomography scan of the abdomen showed diffuse thickening of the ileum (target sign) with mesenteric hypervascularity in a palisading pattern (comb sign), suggesting ischemic ileitis; C, D: Single balloon enteroscopy showed edematous petechiae with linear ulcers in the affected ileum. All figures and legends are reproduced from Hokama et al[15] with permission from BMJ Publishing Group Ltd.

Systemic lupus erythematosus: Systemic lupus erythematosus (SLE) is an autoimmune connective tissue disease with local deposition of antigen-antibody complexes or antibodies inducing necrotizing vasculitis[3].It involves the skin, joints, GI tract, kidneys, central nervous system, and blood cells. Itfrequently involves any part of the GI tract, liver, and pancreas[16,17]. Acute abdominal pain caused by bowel ischemia secondary to lupus mesenteric vasculitis (LMV) is common[18]. The ischemic change can differ according to the sensitivity of the vessels in four different bowel layers; mucosal ulceration and hemorrhage, submucosal edema and intestinal pseudo-obstructiondue to muscular damage, and ascites and perforation due toserosal damage[18]. The endoscopic features areischemic enterocolitis and ‘punched out’ ulcers (Figure 3). Although histopathological diagnosis of LMV can be obtained[19], most endoscopic superficial biopsies might not yield a definitive diagnosis because the affected vessels are usually located in an inaccessible area[18]. The CT features include focal or diffuse bowel wall thickening with the target sign, bowel dilatation, ascites, and engorgement of mesenteric vessels with comb sign (Figure 3)[3,17]. LMV rarely causes pneumatosis intestinalis (PI)[20], which is gas collection in the bowel wall (Figure 4). PI may result in hepatic portal venous gas with a high mortality rate. Another important GI manifestation in SLE is protein losing gastroenteropathy[16]. Edematous villi and lymphangiectasia, which may be caused by immunological vascular or mucosal damage, have been the postulated pathology[20].

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Figure 3 Systemic lupus erythematosus in a 40-year-old woman with lower abdominal pain and fever. A: Contrast-enhanced computed tomography scan of the abdomen showed diffuse thickening of the rectosigmoid colon (black arrow) with engorgement of mesenteric vessels (comb sign, white arrows); B: Colonoscopy disclosed a large punched-out ulcer of the sigmoid colon; C: Perforation of the sigmoid colon occurred despite aggressive immunosuppressive therapy, requiring resection of the affected colon. The resected specimen disclosed bowel perforation with severe transmural inflammation, edema, hemorrhage and vasculitis (hematoxylin-eosin staining, ×40).

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Figure 4 Systemic lupus erythematosus in a 23-year-old woman with abdominal pain and fever. Plain computed tomography scan of the abdomen showed intramural gas of the ascending colon, suggesting pneumatosis intestinalis (arrow). Hyperbaric oxygen therapy was effective for improvement of the pneumatosis.

Behçet’s disease: Behçet’s disease (BD) is a nonspecific necrotizing vasculitis characterized by recurrent orogenital ulcers, uveitis, arthritis, and skinlesions[21,22]. It frequently involves nerves and the GI tract. The frequently involved sites are the ileocecal region and esophagus. The hallmark of BD is the presence of ulceration. Two types of ulceration occur: localized and diffuse[22]. In the ileocecal region, a localized large deeply penetrating ulcer may present with a high frequency of hemorrhage and perforation. The CT features are mass-like lesions and unevenly thickened bowel wall with marked enhancement[3,22]. Barium examination shows a large irregular ulcer with marked thickening of the surrounding intestinal wall (Figure 5). Diffuse lesions are small, multiple, discrete, “punched-out” ulcers commonly observed in the colon (Figure 6)[23]. A recent large scale study confirmed that patients with intestinal BD younger than 25 years, who had a history of prior laparotomy or volcano-shaped intestinal ulcers (the former type) have an increased risk of free bowel perforation[24].

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Figure 5 Behçet’s disease in a 25-year-old woman with abdominal pain and diarrhea. A: Colonoscopy showed a large punched-out ulcer with elevated margins in the terminal ileum; B: Contrast-enhanced computed tomography scan of the abdomen showed a mass-like lesion with unevenly thickened bowel wall of the ileocecal region (arrow); C: Small bowel barium radiography disclosed the large ulcer (arrow) with convergence of mucosal folds in the terminal ileum.

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Figure 6 Behçet’s disease in a 50-year-old woman with abdominal pain and hematochezia-a large ovoid ulcer in the transverse colon. The figure and legends are reproduced from Hokama et al[23] with permission from Elsevier.

Other small-vessel vasculitis: Drugs in nearly all pharmacological classes can cause drug-induced vasculitis/drug-induced lupus-like syndrome[25]. As the clinical presentation and pathological features are indistinguishable from primary vasculitis, a high index of suspicion is required for the accurate diagnosis of drug-induced vasculitis. Discontinuation of the suspected drugis often enough to induce prompt improvement, obviating immunosuppressive treatment.

Infectious agents often cause vasculitis via mechanisms including direct microbial invasion of vascular endothelial cells, immune complex-mediated damage and stimulation of autoreactive lymphocytes through molecular mimicry and superantigens[26]. Causative pathogens include bacteria (e.g., streptococci, mycobacteria, Treponema pallidum), viruses (e.g., cytomegalovirus, herpes virus, hepatitis virus B and C, human immunodeficiency virus), fungi, and parasites.

Vasculitis/connective tissue disease and malignancy are related and this association is bidirectional. Malignancy occurs more frequently in the course of vasculitis and vasculitis occurs in the course of malignancy[27,28]. Therefore, the presence of vasculitis/connective tissue disease may justify a workup for hidden malignancy. In addition, as blood hypercoagulability frequently occurs in malignancy, leading to thrombophlebitis and thrombosis[29], we should pay greater attention to vascular diseases in the treatment of cancer patients.