(Radiographics. 2000;20:779-794.)
© RSNA, 2000
Radiologic Features of Vasculitis Involving the Gastrointestinal Tract1
Hyun K. Ha, MD,
Seung H. Lee, MD,
Sung E. Rha, MD,
Jee-H. Kim, MD,
Jae Y. Byun, MD,
Hyo K. Lim, MD,
Jin W. Chung, MD,
Jeong G. Kim, MD,
Pyo N. Kim, MD,
Moon-G. Lee, MD and
Yong H. Auh, MD
1 From the Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine, 388-1 Poongnap Dong Songpa-Ku, Seoul 138-040, Korea (H.K.H., S.H.L., S.E.R., J.H.K., J.G.K., P.N.K., M.G.K., Y.H.A.); the Department of Radiology, Kangnam St Mary Hospital, Catholic University College of Medicine, Seoul, Korea (J.Y.B.); the Department of Radiology, Samg Sung Medical Center College of Medicine, Sung Kyun Kwan University, Seoul, Korea (H.K.L.); and the Department of Radiology, Seoul National University College of Medicine, Seoul, Korea (J.W.C.). Presented as a scientific exhibit at the 1998 RSNA scientific assembly. Received February 22, 1999; revision requested April 19 and final revision received July 6; accepted July 7. Address reprint requests to H.K.H. (e-mail:
hkha@www.amc.seoul.kr).
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Abstract
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Vasculitides can cause local or diffuse pathologic changes in the gastrointestinal tract, resulting in nonspecific paralytic ileus, mesenteric ischemia, submucosal edema and hemorrhage, or bowel perforation or stricture. The extent and clinical course of disease depend on the size and location of the affected vessel and the histologic characteristics of the lesion. Vasculitis may primarily involve large vessels (eg, giant cell arteritis, Takayasu arteritis), medium-sized vessels (eg, polyarteritis nodosa, Kawasaki disease, primary granulomatous central nervous system vasculitis), or small vessels (eg, Wegener granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, Henoch-Schönlein syndrome, systemic lupus erythematosus, rheumatoid vasculitis, Behçet syndrome). Radiologic findings in various types of vasculitis often overlap considerably and therefore have limited value in making a specific diagnosis. Nevertheless, the possibility of vasculitis should be considered whenever mesenteric ischemic changes occur in young patients, are noted at unusual sites (eg, stomach, duodenum, rectum), have a tendency to concomitantly involve the small and large intestine, and are associated with genitourinary involvement. Knowledge of systemic clinical manifestations in affected patients may suggest and even help establish the specific diagnosis.
Index Terms: Behçet disease, 70.629 • Gastrointestinal tract, angiography, 70.124 • Gastrointestinal tract, CT, 70.1211 • Gastrointestinal tract, inflammation, 70.62 • Gastrointestinal tract, vasculitis, 70.62 • Lupus erythematosus, 70.612 • Vasculitis, 70.62
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Introduction
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Vasculitis can affect blood vessels of all sizes, resulting in necrosis and inflammation. The extent and clinical course of disease depends on the size and location of the affected vessel (1). With larger vessels, abdominal manifestations of vasculitis may be indistinguishable from those of mesenteric ischemia caused by emboli or thrombosis unless there is associated evidence of systemic disease. Inflammation of medium-sized arteries may lead to the formation of aneurysms, which commonly occurs in polyarteritis nodosa. Rupture of these aneurysms may cause gastrointestinal or intraabdominal hemorrhage. With small-vessel involvement, ulceration and stricture formation are common but perforation is seen less frequently. The vasa recta and intramural arteries and arterioles may be affected in virtually all disorders associated with systemic vasculitis.
Until recently, investigators have not sufficiently addressed the role of radiology in distinguishing between ischemia due to mesenteric vasculitides and that due to other causes. It may be difficult to determine the primary causes of gastrointestinal manifestations on the basis of radiologic findings alone. Knowledge of the systemic clinical manifestations in affected patients can suggest the possibility of vasculitis and help establish the specific diagnosis.
In this article, we discuss and illustrate a variety of radiologic findings in 81 patients with vasculitis involving the gastrointestinal tract. Specific disease processes included Behçet disease (n = 34), systemic lupus erythematosus (n = 28), polyarteritis nodosa (n = 7), Henoch-Schönlein purpura (n = 7), microscopic polyangiitis (n = 2), rheumatoid vasculitis (n = 1), Burger disease (n = 1), and Takayasu arteritis (n = 1).
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Classification of Vasculitides
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The vasculitides comprise a diverse group of conditions characterized by inflammation and necrosis of vessel walls. They have many causes and pathogenetic mechanisms, although these are not completely understood. Furthermore, marked variability in clinical manifestation has prevented development of a universally accepted classification of vasculitis. Several investigators have attempted to classify vasculitis on the basis of differing aspects of the disease such as extent or location of involvement, pathologic characteristics, pathogenetic processes, or clinical symptoms (2–5). In 1990, the American College of Rheumatology published an approach to classifying vasculitides for clinical trials in an attempt to establish the clinical findings that are helpful in identifying vasculitis and distinguishing it from other diseases (3). However, this study was limited to seven forms of vasculitis that are usually considered to be clinically distinct: polyarteritis nodosa, Churg-Strauss syndrome, Wegener granulomatosis, hypersensitivity vasculitis, Henoch-Schönlein syndrome, giant cell (temporal) arteritis, and Takayasu arteritis. In 1994, the Chapel Hill International Consensus Conference proposed names and definitions for selected categories of vasculitis (2). The authors classified 10 selected vasculitides as large-vessel vasculitis, medium-sized vessel vasculitis, and small-vessel vasculitis, depending on the type of vessel that is predominantly affected. Large-vessel vasculitis affects the aorta and the largest arterial branches directed toward major body regions, medium-sized vessel vasculitis affects the main visceral arteries and their branches, and small-vessel vasculitis affects arterioles, venules, and capillaries (Table). Although there remains substantial overlap among the different vasculitides, this classification system helps establish the concept of "small-vessel vasculitides" as distinct from vasculitides that predominantly affect medium-sized and large arteries (Fig 1). However, this classification system assigns microscopic polyangiitis too prominent a place in the family of vasculitides, and other clinical, biologic, and histologic criteria are not taken into consideration. The ability to identify pathogenic antibodies in tissue and serum led to discoveries that allowed the categorization of certain small-vessel vasculitides. Recently identified ANCAs are closely associated with three major categories of small-vessel vasculitis with absence or paucity of vascular immune deposits, such as Wegener granulomatosis, microscopic polyangiitis (polyarteritis), and Churg-Strauss syndrome (6). However, assays for ANCA are negative in approximately 10% of patients with typical Wegener granulomatosis or microscopic polyangiitis, and the specificity of a positive ANCA assay is not absolute. Immune complexes are identified in certain types of vasculitis, including Henoch-Schönlein purpura, lupus vasculitis, Behçet disease, and others (6).
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Figure 1. Drawing illustrates the preferred sites of vascular involvement by selected vasculitides. The widths of the trapezoids indicate the frequency of involvement of various portions of the vasculature. LCA = leukocytoclastic angiitis. (Reprinted, with permission, from reference 6.)
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Large-Vessel Vasculitis (Takayasu Arteritis)
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Takayasu arteritis is a form of granulomatous vasculitis affecting large and medium-sized arteries that is characterized by ocular disturbances and marked weakening of the pulses in the upper extremities (pulseless disease). It is also associated with fibrous thickening of the aortic arch with narrowing or virtual obliteration of the origins of the great vessels arising in the arch. Although Takayasu arteritis classically involves the aortic arch, in 32% of cases it also affects the remainder of the aorta and its branches and in 12% it is limited to the descending thoracic and abdominal aorta (1) (Fig 2). In most cases, the gross morphologic changes consist of irregular thickening of the aortic wall with intimal wrinkling. In general, the possibility of underlying arteritis should be considered when arteriosclerotic changes in the aorta are seen in young or middle-aged patients, especially Asian and female patients, and when these changes are either segmental or occur at an unusual site. Generalized signs and symptoms include malaise, fever, night sweats, arthralgia, and weight loss. Pulses are commonly absent in the involved vessels, particularly the subclavian artery. The diagnosis is confirmed by a characteristic arteriographic pattern that includes irregular vessel walls, stenosis, poststenotic dilatation, aneurysm formation, occlusion, and evidence of increased collateral circulation.
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Figure 2a. Takayasu arteritis in a 26-year-old woman who demonstrated coldness and weak pulses in both hands and feet and bruit in the areas of the neck and abdomen at physical examination. (a) Arteriogram shows luminal narrowing of the proximal superior mesenteric artery (curved arrows) with a prominent arc of Riolan (arrowheads). Irregular narrowing of the superior left colic branches of the inferior mesenteric artery is also noted (straight arrows). (b) Aortogram of the aortic arch shows nearly complete occlusion of both subclavian arteries (arrows) with development of collateral circulation.
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Figure 2b. Takayasu arteritis in a 26-year-old woman who demonstrated coldness and weak pulses in both hands and feet and bruit in the areas of the neck and abdomen at physical examination. (a) Arteriogram shows luminal narrowing of the proximal superior mesenteric artery (curved arrows) with a prominent arc of Riolan (arrowheads). Irregular narrowing of the superior left colic branches of the inferior mesenteric artery is also noted (straight arrows). (b) Aortogram of the aortic arch shows nearly complete occlusion of both subclavian arteries (arrows) with development of collateral circulation.
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Medium-sized-Vessel Vasculitis (Polyarteritis Nodosa)
Polyarteritis nodosa is a fibrinoid necrotizing vasculitis that mainly involves small and medium-sized arteries of the muscles. Multiple aneurysm formation is a characteristic finding in polyarteritis nodosa (50%–60% of cases). Lesions are caused by segmental erosion with weakening of the arterial wall due to the inflammatory process and affect the branch points and bifurcation of arteries. The kidney is most commonly involved (80%–90% of cases), followed by the gastrointestinal tract (50%–70%), liver (50%–60%), spleen (45%), and pancreas (25%–35%) (Figs 3, 4) (8–10). The small intestine is the most commonly affected portion of the gastrointestinal tract, followed by the mesentery and colon. Patients usually present with vague signs and symptoms such as fever, weight loss, weakness, malaise, headache, and myalgia. Specific complaints related to vascular involvement within a particular organ system include renal failure or hypertension in renal involvement; peripheral neuropathy in peripheral nervous system involvement; congestive heart failure, myocardial infarction, or pericarditis in cardiac involvement; and rash, purpura, or nodules in skin involvement (9). Approximately two-thirds of patients with polyarteritis nodosa have abdominal pain, nausea, vomiting, or other gastrointestinal signs and symptoms in association with organ damage due to ischemia and infarction; gastrointestinal hemorrhage occurs in roughly 6% of cases, bowel perforation in 5%, and bowel infarction in 1.4% (8,11). There are no diagnostic serologic tests for classic polyarteritis nodosa. Hypergammaglobulinemia may be present, and up to 30% of patients test positive for hepatitis B surface antigen (9). Positive ANCA titers (usually of the perinuclear ANCA type) are found in variable percentages of patients. If left untreated, the disease is fatal in most instances in association with renal failure, gastrointestinal complications such as bowel infarction or perforation, or cardiovascular causes (12). However, therapy with corticosteroids and cyclophosphamide results in remission or cure in 90% of affected patients (1).
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Figure 3a. Polyarteritis nodosa in a 39-year-old man with a 3-month history of fever, myalgia, arthralgia, and peripheral neuropathy. (a) Contrast material-enhanced computed tomographic (CT) scan shows bowel wall thickening of the ileum and ascending and descending colon (arrows) with evidence of the target sign. Extensive ascites and bilateral ureteral dilatation are also noted (arrowheads). (b) Contrast-enhanced CT scan shows rectal wall thickening (arrowheads) with the target sign. The bladder (B) is also diffusely thickened. (c) Aortogram shows multiple microaneurysms in the branches of the superior mesenteric (arrows) and hepatic (arrowheads) arteries. The diagnosis of polyarteritis nodosa was made at angiography.
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Figure 3b. Polyarteritis nodosa in a 39-year-old man with a 3-month history of fever, myalgia, arthralgia, and peripheral neuropathy. (a) Contrast material-enhanced computed tomographic (CT) scan shows bowel wall thickening of the ileum and ascending and descending colon (arrows) with evidence of the target sign. Extensive ascites and bilateral ureteral dilatation are also noted (arrowheads). (b) Contrast-enhanced CT scan shows rectal wall thickening (arrowheads) with the target sign. The bladder (B) is also diffusely thickened. (c) Aortogram shows multiple microaneurysms in the branches of the superior mesenteric (arrows) and hepatic (arrowheads) arteries. The diagnosis of polyarteritis nodosa was made at angiography.
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Figure 3c. Polyarteritis nodosa in a 39-year-old man with a 3-month history of fever, myalgia, arthralgia, and peripheral neuropathy. (a) Contrast material-enhanced computed tomographic (CT) scan shows bowel wall thickening of the ileum and ascending and descending colon (arrows) with evidence of the target sign. Extensive ascites and bilateral ureteral dilatation are also noted (arrowheads). (b) Contrast-enhanced CT scan shows rectal wall thickening (arrowheads) with the target sign. The bladder (B) is also diffusely thickened. (c) Aortogram shows multiple microaneurysms in the branches of the superior mesenteric (arrows) and hepatic (arrowheads) arteries. The diagnosis of polyarteritis nodosa was made at angiography.
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Figure 4a. Polyarteritis nodosa in a 42-year-old man with a 1-month history of fever, lower extremity pain, hematuria, and right flank pain. (a) Contrast-enhanced CT scan shows renal and perinephric hematoma (H) along with multifocal areas of wedge-shaped defects in both renal parenchyma due to infarcts. (b) Contrast-enhanced CT scan shows focal bowel wall thickening of the jejunum (arrowheads) with diffuse gaseous intestinal dilatation. Bilateral ureteral dilatation is also noted (arrows). (c) Angiogram of the right kidney shows multiple aneurysms in the renal arterial branches (arrowheads) with renal parenchymal defects due to renal and perinephric hematoma (arrows). (d) Hepatic angiogram shows numerous microaneurysms in the hepatic arterial branches (arrowheads). (e) Arteriogram shows microaneurysms in the superior mesenteric arterial branches (arrows). The diagnosis of polyarteritis nodosa was made at skin biopsy and angiography.
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Figure 4b. Polyarteritis nodosa in a 42-year-old man with a 1-month history of fever, lower extremity pain, hematuria, and right flank pain. (a) Contrast-enhanced CT scan shows renal and perinephric hematoma (H) along with multifocal areas of wedge-shaped defects in both renal parenchyma due to infarcts. (b) Contrast-enhanced CT scan shows focal bowel wall thickening of the jejunum (arrowheads) with diffuse gaseous intestinal dilatation. Bilateral ureteral dilatation is also noted (arrows). (c) Angiogram of the right kidney shows multiple aneurysms in the renal arterial branches (arrowheads) with renal parenchymal defects due to renal and perinephric hematoma (arrows). (d) Hepatic angiogram shows numerous microaneurysms in the hepatic arterial branches (arrowheads). (e) Arteriogram shows microaneurysms in the superior mesenteric arterial branches (arrows). The diagnosis of polyarteritis nodosa was made at skin biopsy and angiography.
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Figure 4c. Polyarteritis nodosa in a 42-year-old man with a 1-month history of fever, lower extremity pain, hematuria, and right flank pain. (a) Contrast-enhanced CT scan shows renal and perinephric hematoma (H) along with multifocal areas of wedge-shaped defects in both renal parenchyma due to infarcts. (b) Contrast-enhanced CT scan shows focal bowel wall thickening of the jejunum (arrowheads) with diffuse gaseous intestinal dilatation. Bilateral ureteral dilatation is also noted (arrows). (c) Angiogram of the right kidney shows multiple aneurysms in the renal arterial branches (arrowheads) with renal parenchymal defects due to renal and perinephric hematoma (arrows). (d) Hepatic angiogram shows numerous microaneurysms in the hepatic arterial branches (arrowheads). (e) Arteriogram shows microaneurysms in the superior mesenteric arterial branches (arrows). The diagnosis of polyarteritis nodosa was made at skin biopsy and angiography.
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Figure 4d. Polyarteritis nodosa in a 42-year-old man with a 1-month history of fever, lower extremity pain, hematuria, and right flank pain. (a) Contrast-enhanced CT scan shows renal and perinephric hematoma (H) along with multifocal areas of wedge-shaped defects in both renal parenchyma due to infarcts. (b) Contrast-enhanced CT scan shows focal bowel wall thickening of the jejunum (arrowheads) with diffuse gaseous intestinal dilatation. Bilateral ureteral dilatation is also noted (arrows). (c) Angiogram of the right kidney shows multiple aneurysms in the renal arterial branches (arrowheads) with renal parenchymal defects due to renal and perinephric hematoma (arrows). (d) Hepatic angiogram shows numerous microaneurysms in the hepatic arterial branches (arrowheads). (e) Arteriogram shows microaneurysms in the superior mesenteric arterial branches (arrows). The diagnosis of polyarteritis nodosa was made at skin biopsy and angiography.
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Figure 4e. Polyarteritis nodosa in a 42-year-old man with a 1-month history of fever, lower extremity pain, hematuria, and right flank pain. (a) Contrast-enhanced CT scan shows renal and perinephric hematoma (H) along with multifocal areas of wedge-shaped defects in both renal parenchyma due to infarcts. (b) Contrast-enhanced CT scan shows focal bowel wall thickening of the jejunum (arrowheads) with diffuse gaseous intestinal dilatation. Bilateral ureteral dilatation is also noted (arrows). (c) Angiogram of the right kidney shows multiple aneurysms in the renal arterial branches (arrowheads) with renal parenchymal defects due to renal and perinephric hematoma (arrows). (d) Hepatic angiogram shows numerous microaneurysms in the hepatic arterial branches (arrowheads). (e) Arteriogram shows microaneurysms in the superior mesenteric arterial branches (arrows). The diagnosis of polyarteritis nodosa was made at skin biopsy and angiography.
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The diagnosis of polyarteritis nodosa is suggested by an angiographic finding of aneurysms up to 1 cm in diameter within the renal, mesenteric, and hepatic vasculature; however, this finding is not always pathognomonic for polyarteritis nodosa because it is also seen in necrotizing angiitis associated with drug abuse (13) and other vasculitides such as Wegener granulomatosis and systemic lupus erythematosus (9). Therefore, radiologic findings, clinical symptoms, and pathologic findings that are consistent with fibrinoid necrotizing vasculitis are necessary to diagnose polyarteritis nodosa.
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Small-Vessel Vasculitis
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Wegener Granulomatosis
Wegener granulomatosis is a distinct clinicopathologic entity characterized by granulomatous vasculitis of the upper and lower respiratory tract together with glomerulonephritis. Wegener granulomatosis may involve any part of the gastrointestinal tract. In one series of 45 patients, 10% had gastrointestinal signs and symptoms such as abdominal pain, diarrhea, and blood loss at initial presentation or during relapse (14). In a review of 56 autopsy cases, focal necrotizing arteriolitis was found in the intestine in 24% (15). However, clinical manifestations of severe intestinal disease are rare and appear to be associated with the disease process rather than with the use of immunosuppressive agents (16). Histologic examination of resected intestinal material in patients with severe gastrointestinal involvement demonstrated vasculitis, ischemia, inflammation, and ulceration in both the small and large intestine (16), and bowel perforations appear to be the most common cause of acute abdomen. On rare occasions, Wegener granulomatosis mimics inflammatory bowel disease (eg, Crohn disease, ulcerative colitis) (17).
Churg-Strauss Syndrome
Churg-Strauss syndrome is characterized by granulomatous vasculitis of multiple organ systems, particularly the lung, and involves both arteries and veins as well as pulmonary and systemic vessels. Churg-Strauss syndrome has three phases: allergic rhinitis and asthma; peripheral blood and tissue eosinophilia with Löffler syndrome, chronic eosinophilic pneumonia, or eosinophilic gastroenteritis; and systemic small-vessel vasculitis with granulomatous inflammation (6). General signs and symptoms such as fever or weight loss are seen in most patients, and their development in patients with asthma is suggestive of the diagnosis. Extrapulmonary lesions are more commonly found in the gastrointestinal tract (20% of cases), spleen, and heart than in the kidney. Gastrointestinal involvement typically manifests as intestinal inflammation, ulceration, perforation, or hemorrhage (18). Two mechanisms of gastrointestinal involvement are possible: Mesenteric vasculitis is the most common, whereas bowel wall infiltration by eosinophils is rare and may cause obstructive symptoms or diarrhea and bleeding (19).
Microscopic Polyangiitis
Microscopic polyangiitis (microscopic polyarteritis, hypersensitivity vasculitis, leukocytoclastic vasculitis) is characterized by pauciimmune, necrotizing small-vessel vasculitis without clinical or pathologic evidence of necrotizing granulomatous inflammation (2). At histologic analysis, this disease is identical to polyarteritis nodosa except for the presence of vasculitis in vessels smaller than arteries (ie, arterioles, venules, capillaries) (6). Most patients with suspected hypersensitivity vasculitis prove to have microscopic polyangiitis or cutaneous leukocytoclastic angiitis (2). At the 1994 Chapel Hill International Consensus Conference, the term hypersensitivity vasculitis was not used; instead, use of the term microscopic angiitis was advocated (2). ANCAs are present in the majority of cases.
The lesions are thought to represent a hypersensitivity reaction and involve the skin, mucous membranes, lung, brain, heart, gastrointestinal tract, kidneys, and muscle. Necrotizing glomerulonephritis (seen in 90% of cases) and pulmonary capillaritis are particularly common. Clinical manifestations include hemoptysis, hematuria, proteinuria, abdominal pain or gastrointestinal bleeding, and muscle pain or weakness. In many instances, the lesions are limited to the skin (cutaneous leukocytoclastic vasculitis). Drugs (eg, penicillin), micro-organisms, heterologous proteins, and tumor antigens have been implicated as triggers of this disorder (1). With the exception of those who develop widespread renal or brain lesions, most patients respond well to simple removal of the offending agent. When the gastrointestinal tract is involved (Figs 5, 6), radiologic findings do not differ from those seen in other types of vasculitis involving similar-sized vessels, and bowel infarct or perforation rarely occurs (20,21). Unlike with polyarteritis nodosa, findings at visceral angiography in microscopic polyangiitis are usually normal and do not reveal microaneurysms.
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Figure 5a. Microscopic polyangiitis (hypersensitivity vasculitis) in a 44-year-old man who had been treated for a benign esophageal stricture of unknown origin and developed sudden onset of abdominal pain, multiple joint pain, oliguria, and skin pustules following antibiotic therapy with cephalosporin. (a) Contrast-enhanced CT scan shows duodenal wall thickening (arrows) with ascites. (b) Contrast-enhanced CT scan shows bowel wall thickening of the ileum (arrows) with the target sign. Limited ascites is also noted. The diagnosis of microscopic polyangiitis was made at skin biopsy.
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Figure 5b. Microscopic polyangiitis (hypersensitivity vasculitis) in a 44-year-old man who had been treated for a benign esophageal stricture of unknown origin and developed sudden onset of abdominal pain, multiple joint pain, oliguria, and skin pustules following antibiotic therapy with cephalosporin. (a) Contrast-enhanced CT scan shows duodenal wall thickening (arrows) with ascites. (b) Contrast-enhanced CT scan shows bowel wall thickening of the ileum (arrows) with the target sign. Limited ascites is also noted. The diagnosis of microscopic polyangiitis was made at skin biopsy.
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Figure 6a. Microscopic polyangiitis (hypersensitivity vasculitis) with bowel infarct in a 30-year-old man with a 2-week history of diffuse abdominal pain. (a) Contrast-enhanced CT scan shows a thrombus in the proximal superior mesenteric vein (arrow). (b) Contrast-enhanced CT scan shows bowel wall thickening of the jejunum (arrows) as well as evidence of regional mesenteric vascular engorgement. The diagnosis of microscopic polyangiitis was made at histopathologic examination of the resected specimen following surgery. (Fig 6 reprinted, with permission, from reference 20.)
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Figure 6b. Microscopic polyangiitis (hypersensitivity vasculitis) with bowel infarct in a 30-year-old man with a 2-week history of diffuse abdominal pain. (a) Contrast-enhanced CT scan shows a thrombus in the proximal superior mesenteric vein (arrow). (b) Contrast-enhanced CT scan shows bowel wall thickening of the jejunum (arrows) as well as evidence of regional mesenteric vascular engorgement. The diagnosis of microscopic polyangiitis was made at histopathologic examination of the resected specimen following surgery. (Fig 6 reprinted, with permission, from reference 20.)
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Henoch-Schönlein Syndrome
Henoch-Schönlein syndrome is one of the subgroups of hypersensitivity-related acute small-vessel vasculitis (22,23). Although its cause is unknown, immunization, insect bites, medications, infections, and certain foods may play a role in its development. Henoch-Schönlein syndrome occurs most commonly in children between 3 and 10 years of age, but in some series, 30% of affected patients have been reported to be over 20 years old (22,23). The diagnosis is based on characteristic clinical signs and symptoms such as skin rash, arthritis involving the large joints, colicky abdominal pain, gastrointestinal bleeding, and hematuria (23). The gastrointestinal manifestations are thought to be related to edema and intramural hemorrhage (Fig 7). When gastrointestinal symptoms predominate or precede the appearance of skin lesions, the syndrome may mimic a number of acute abdominal diseases, resulting in unnecessary laparotomies. Gastrointestinal hemorrhage is mostly confined to the mucosa and submucosa, and full-thickness necrosis and perforation of a bowel loop is rare. Therefore, most gastrointestinal manifestations are self-limited and without residua: Only 3%–5% of patients develop bowel infarct (Fig 8), perforation, or irreducible intussusception (24). No characteristic radiologic findings are seen, although multifocal bowel wall thickening with unaffected areas along with clinical findings are important in establishing a diagnosis (25).
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Figure 7. Henoch-Schönlein purpura in a 5-year-old girl who presented with skin rash, arthralgia, and mild abdominal pain. Contrast-enhanced CT scan shows heterogeneous bowel wall thickening of the ileum (arrows).
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Figure 8a. Henoch-Schönlein purpura with bowel infarct in a 6-year-old boy who presented with severe colicky abdominal pain, vomiting, and polyarthralgia and had purpura on both buttocks at physical examination. (a) Contrast-enhanced CT scan shows bowel wall thickening of the ileum (*) with the target sign. Ascites is also noted. (b) Image from a small bowel follow-through study shows luminal narrowing and circular fold thickening in both the jejunum (black arrowheads) and ileum (white arrowheads) with unaffected areas. The diagnosis of Henoch-Schönlein purpura was made at histopathologic analysis following surgery (segmental ileal resection).
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Figure 8b. Henoch-Schönlein purpura with bowel infarct in a 6-year-old boy who presented with severe colicky abdominal pain, vomiting, and polyarthralgia and had purpura on both buttocks at physical examination. (a) Contrast-enhanced CT scan shows bowel wall thickening of the ileum (*) with the target sign. Ascites is also noted. (b) Image from a small bowel follow-through study shows luminal narrowing and circular fold thickening in both the jejunum (black arrowheads) and ileum (white arrowheads) with unaffected areas. The diagnosis of Henoch-Schönlein purpura was made at histopathologic analysis following surgery (segmental ileal resection).
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Systemic Lupus Erythematosus
Systemic lupus erythematosus is an autoimmune disorder that affects the musculoskeletal system, kidneys, gastrointestinal tract, or skin (26). Local deposition of antigen-antibody complexes or antibodies inducing necrotizing vasculitis is presumed to be the cause of this disease. Most patients are between 16 and 41 years old, with the disease occurring most commonly in women during the child-bearing years. A diagnosis of systemic lupus erythematosus can be made with 98% specificity and 97% sensitivity if at least four of 11 diagnostic criteria are present at any time during the course of the disease (27). These criteria include malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, antinuclear antibodies, and renal, neurologic, hematologic, and immunologic disorders. Inflammation of the small blood vessels of the gut produces a variety of complications including intestinal ischemia, hemorrhage, ileus, ulceration, infarction, and perforation (28). Systemic lupus erythematosus may involve any part of the gastrointestinal tract from the esophagus to the colon including the stomach (Fig 9), duodenum, colon, and rectum. However, the territory of the superior mesenteric artery is most commonly affected.
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Figure 9a. Systemic lupus erythematosus in a 37-year-old woman who presented with fever, cough, abdominal pain, polyarthralgia, and skin rash. The patient tested positive for antinuclear antibodies. (a) Contrast-enhanced CT scan shows diffuse gastric wall thickening (arrows) with the target sign. (b) Contrast-enhanced CT scan shows a similar pattern of bowel wall thickening in the duodenum (arrows) and jejunum (arrowheads) with unaffected segments. The diagnosis of systemic lupus erythematosus was made at renal biopsy.
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Figure 9b. Systemic lupus erythematosus in a 37-year-old woman who presented with fever, cough, abdominal pain, polyarthralgia, and skin rash. The patient tested positive for antinuclear antibodies. (a) Contrast-enhanced CT scan shows diffuse gastric wall thickening (arrows) with the target sign. (b) Contrast-enhanced CT scan shows a similar pattern of bowel wall thickening in the duodenum (arrows) and jejunum (arrowheads) with unaffected segments. The diagnosis of systemic lupus erythematosus was made at renal biopsy.
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CT allows more accurate diagnosis of mesenteric arteritis. Common CT findings include dilated bowel, focal or diffuse bowel wall thickening, abnormal bowel wall enhancement, engorged mesenteric vessels in a comblike arrangement (comb sign), ascites, and lymphadenopathy (Fig 10) (29). Bowel wall thickening is usually multifocal and not confined to a single vascular territory because mesenteric vasculitis may affect several vessels simultaneously (30). Rather than bowel wall changes, the ancillary findings occurring in other organs may suggest the diagnosis because a high prevalence of genitourinary tract involvement (lupus nephritis, cystitis, hydronephrosis) has been reported (Fig 11) (29). Hydronephrosis also occurs in other types of vasculitis such as polyarteritis nodosa and Henoch-Schönlein syndrome and seems to be caused either by detrusor muscle spasm with subsequent vesicoureteral reflux or by fibrosis of the ureterovesical junction (31–33).
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Figure 10a. Systemic lupus erythematosus in a 26-year-old woman who complained of sudden onset of abdominal pain, diarrhea, and vomiting and met the clinical diagnostic criteria for this disorder. (a) Contrast-enhanced CT scan shows bowel wall thickening of the third and fourth portions of the duodenum (*) with the target sign. Splenomegaly (S) and limited ascites are also noted. (b) Contrast-enhanced CT scan demonstrates bowel wall thickening of the small intestine with the target sign. Mesenteric vascular engorgement (arrowheads) and extensive ascites are also noted.
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Figure 10b. Systemic lupus erythematosus in a 26-year-old woman who complained of sudden onset of abdominal pain, diarrhea, and vomiting and met the clinical diagnostic criteria for this disorder. (a) Contrast-enhanced CT scan shows bowel wall thickening of the third and fourth portions of the duodenum (*) with the target sign. Splenomegaly (S) and limited ascites are also noted. (b) Contrast-enhanced CT scan demonstrates bowel wall thickening of the small intestine with the target sign. Mesenteric vascular engorgement (arrowheads) and extensive ascites are also noted.
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Figure 11. Systemic lupus erythematosus in a 32-year-old woman who presented with abdominal pain, diarrhea, and fever and met the clinical diagnostic criteria for this disorder. Contrast-enhanced CT scan shows heterogeneous bowel wall thickening of the jejunum (arrowheads) and ascending and descending colon (solid arrows). Bilateral ureteral dilatation is also noted (open arrows).
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Rheumatoid Vasculitis
Rheumatoid vasculitis is classically a leukocytoclastic lesion of small venules that predominantly involves the skin. The disease is probably due to immune complexes, which have been identified in the walls of involved vessels (34). Rarely, this disease may cause life-threatening visceral infarction (Fig 12) (1). Rheumatoid vasculitis occurs predominantly after long-standing rheumatoid arthritis in patients who also exhibit rheumatoid nodules, hypocomplementemia, and high titers of rheumatoid factor (1). However, it is well known that gastrointestinal symptoms and damage result from the use of nonsteroidal, steroidal, and anti-inflammatory and immunosuppressive medications (35). These drugs can affect the mouth, esophagus, stomach, intestine, liver, and pancreas. Therefore, if gastrointestinal symptoms develop in patients with long-standing rheumatoid arthritis, it may be difficult to determine whether the patient's symptoms originate from the disease itself or from ongoing use of medications. In addition to the changes in the musculoskeletal system, nonspecific inflammatory changes or rheumatoid nodules may be found in the lungs, pleura, pericardium, and peripheral nerves. Neurovascular disease manifesting either as a mild, distal sensory neuropathy or as mononeuritis multiplex may be the only sign of vasculitis (36). The use of cytotoxic immunosuppressive drugs is justified in patients with severe extraarticular manifestations of disease (36).
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Figure 12. Rheumatoid vasculitis involving the mesenteric vessels in a 49-year-old man with a 3-day history of epigastric pain, vomiting, diarrhea, and general weakness and a 10-year history of rheumatoid arthritis. Contrast-enhanced CT scan shows diffuse bowel wall thickening of the small (S) and large (*) intestine with the target sign.
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Behçet Syndrome
Behçet syndrome is a widely recognized, nonspecific necrotizing vasculitis involving multiple organ systems with well-known clinical manifestations including orogenital ulceration, uveitis, arthritis, and neurologic and gastrointestinal involvement (37,38). The gastrointestinal tract has been reported to be involved in 10%–40% of patients with Behçet syndrome (39); the usual site is the ileocecal area, especially the terminal ileum. The hallmark of Behçet syndrome is the presence of large, deeply penetrating ulcerations of the submucosa, muscle layer, or entire intestinal wall. As a result, a high prevalence of complications such as perforation, hemorrhage, fistula, and peritonitis has been reported (40). Appropriate treatment is of great importance in avoiding these complications.
Barium examination shows a large ovoid or irregular ulcer with marked thickening of the surrounding intestinal wall at the sites of involvement. In some cases, however, small, multiple, discrete, "punched-out" ulcers can be seen. Recently, CT proved to be effective not only for detecting primary lesions but also for excluding extraluminal complications (41). At CT, the involved bowel demonstrates concentric bowel wall thickening or a polypoid mass. Interestingly, most of the thickened bowel wall demonstrates marked contrast enhancement (Figs 13, 14) (41). In patients with no complications, perienteric or pericolonic infiltration is absent or minimal. In contrast, the presence of severe infiltration raises the possibility of complications such as microperforation or localized peritonitis (Fig 15).
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Figure 13a. Behçet syndrome in a 42-year-old man with oral and genital ulcers, uveitis, right lower quadrant pain, and arthralgia at the knee and elbow. (a) Image from a double contrast barium enema study shows a large, irregular ulcer in the terminal ileum (arrow) with convergence of thickened mucosal folds toward the ulcer (arrowheads). (b) Contrast-enhanced CT scan shows bowel wall thickening with marked enhancement (*). Perienteric infiltration is absent or very minimal. Colonoscopic biopsy showed necrotizing vasculitis at the lesion site.
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Figure 13b. Behçet syndrome in a 42-year-old man with oral and genital ulcers, uveitis, right lower quadrant pain, and arthralgia at the knee and elbow. (a) Image from a double contrast barium enema study shows a large, irregular ulcer in the terminal ileum (arrow) with convergence of thickened mucosal folds toward the ulcer (arrowheads). (b) Contrast-enhanced CT scan shows bowel wall thickening with marked enhancement (*). Perienteric infiltration is absent or very minimal. Colonoscopic biopsy showed necrotizing vasculitis at the lesion site.
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Figure 14a. Behçet syndrome in a 39-year-old man with an oral ulcer and right lower quadrant pain. (a) Image from a double contrast barium enema study shows multiple discrete ulcers (arrows) with thickened mucosal folds at the terminal ileum. (b) Contrast-enhanced CT scan shows bowel wall thickening (arrows) and a polypoid masslike lesion (arrowheads) in the ileocecal region. Colonoscopic biopsy revealed necrotizing vasculitis.
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Figure 14b. Behçet syndrome in a 39-year-old man with an oral ulcer and right lower quadrant pain. (a) Image from a double contrast barium enema study shows multiple discrete ulcers (arrows) with thickened mucosal folds at the terminal ileum. (b) Contrast-enhanced CT scan shows bowel wall thickening (arrows) and a polypoid masslike lesion (arrowheads) in the ileocecal region. Colonoscopic biopsy revealed necrotizing vasculitis.
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Figure 15a. Behçet syndrome in a 32-year-old woman who presented with diffuse abdominal pain and tenderness. The patient had a history of recurrent oral and genital ulcers and uveitis and of segmental resection of the ileum due to ulcer perforation that had occurred 2 months earlier. (a) Contrast-enhanced CT scan shows marked enhancement of the thickened bowel wall in the distal ileum (curved arrow) with evidence of diffuse infiltration in the regional mesentery (straight arrows). (b) Photograph of a gross specimen from a right hemicolectomy with resection of the distal ileum (T) shows discrete, "punched-out" ulcers (arrowheads) as well as irregular perforations (arrows) in both the small and large intestine. C = cecum. (Reprinted, with permission, from reference 41.)
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Figure 15b. Behçet syndrome in a 32-year-old woman who presented with diffuse abdominal pain and tenderness. The patient had a history of recurrent oral and genital ulcers and uveitis and of segmental resection of the ileum due to ulcer perforation that had occurred 2 months earlier. (a) Contrast-enhanced CT scan shows marked enhancement of the thickened bowel wall in the distal ileum (curved arrow) with evidence of diffuse infiltration in the regional mesentery (straight arrows). (b) Photograph of a gross specimen from a right hemicolectomy with resection of the distal ileum (T) shows discrete, "punched-out" ulcers (arrowheads) as well as irregular perforations (arrows) in both the small and large intestine. C = cecum. (Reprinted, with permission, from reference 41.)
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Miscellaneous (Thromboangiitis Obliterans)
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Thromboangiitis obliterans (Burger disease) is a distinctive disease characterized by segmental, thrombosing, acute or chronic inflammation of small and intermediate-sized arteries and veins of the extremities. Although it is not a classic vasculitis, this disease is considered to be one of the vasculitides due to the intense inflammatory response within the thrombus and the frequent manifestation of vasculitis of the vasa vasorum in the arterial wall. Thromboangiitis obliterans occurs almost exclusively in smokers and begins before the age of 35 years. Remission and relapse correlate with cessation or resumption of smoking. Thromboangiitis obliterans is often associated with migratory thrombophlebitis but is not associated with diabetes mellitus, hypercholesterolemia, or heart disease (1). Unlike with arteriosclerosis, arterial calcification is absent.
Although rare, a form of thromboangiitis obliterans that preferentially involves the mesenteric vessels has also been described in the literature (Fig 16) (42). Since 1945, 17 cases of this rare form of thromboangiitis obliterans have been reported. In 12 cases, peripheral arterial disease preceded the onset of abdominal symptoms; in three cases, it appeared later in the course of the disease; and in two cases, it was not seen at all. The small intestine was more commonly affected than the large intestine.
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Figure 16a. Thromboangiitis obliterans in a 31-year-old man who presented with complaints of bluish discoloration and tenderness of the toes. The patient had been a smoker (1 pack per day) for 10 years. (a) Angiogram shows severe luminal narrowing of the proximal superior mesenteric artery (arrow). Subtraction artifact is seen mimicking distal superior mesenteric arterial occlusion (arrowhead). (b) Aortogram shows a prominent marginal artery of Drummond (arrows). (c) Inferior mesenteric angiogram shows a prominent marginal artery of Drummond (arrowheads) with depiction of superior mesenteric arterial branches. (d) Angiogram of both lower extremities shows occlusion of the right anterior tibial artery (AT), luminal narrowing of the right peroneal artery (PN), and evidence of irregular luminal narrowing of the left anterior (AT) and posterior (PT) tibial arteries.
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Figure 16b. Thromboangiitis obliterans in a 31-year-old man who presented with complaints of bluish discoloration and tenderness of the toes. The patient had been a smoker (1 pack per day) for 10 years. (a) Angiogram shows severe luminal narrowing of the proximal superior mesenteric artery (arrow). Subtraction artifact is seen mimicking distal superior mesenteric arterial occlusion (arrowhead). (b) Aortogram shows a prominent marginal artery of Drummond (arrows). (c) Inferior mesenteric angiogram shows a prominent marginal artery of Drummond (arrowheads) with depiction of superior mesenteric arterial branches. (d) Angiogram of both lower extremities shows occlusion of the right anterior tibial artery (AT), luminal narrowing of the right peroneal artery (PN), and evidence of irregular luminal narrowing of the left anterior (AT) and posterior (PT) tibial arteries.
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Figure 16c. Thromboangiitis obliterans in a 31-year-old man who presented with complaints of bluish discoloration and tenderness of the toes. The patient had been a smoker (1 pack per day) for 10 years. (a) Angiogram shows severe luminal narrowing of the proximal superior mesenteric artery (arrow). Subtraction artifact is seen mimicking distal superior mesenteric arterial occlusion (arrowhead). (b) Aortogram shows a prominent marginal artery of Drummond (arrows). (c) Inferior mesenteric angiogram shows a prominent marginal artery of Drummond (arrowheads) with depiction of superior mesenteric arterial branches. (d) Angiogram of both lower extremities shows occlusion of the right anterior tibial artery (AT), luminal narrowing of the right peroneal artery (PN), and evidence of irregular luminal narrowing of the left anterior (AT) and posterior (PT) tibial arteries.
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Figure 16d. Thromboangiitis obliterans in a 31-year-old man who presented with complaints of bluish discoloration and tenderness of the toes. The patient had been a smoker (1 pack per day) for 10 years. (a) Angiogram shows severe luminal narrowing of the proximal superior mesenteric artery (arrow). Subtraction artifact is seen mimicking distal superior mesenteric arterial occlusion (arrowhead). (b) Aortogram shows a prominent marginal artery of Drummond (arrows). (c) Inferior mesenteric angiogram shows a prominent marginal artery of Drummond (arrowheads) with depiction of superior mesenteric arterial branches. (d) Angiogram of both lower extremities shows occlusion of the right anterior tibial artery (AT), luminal narrowing of the right peroneal artery (PN), and evidence of irregular luminal narrowing of the left anterior (AT) and posterior (PT) tibial arteries.
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Conclusions
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Radiologic findings in various types of vasculitis often overlap considerably, although the possibility of this disease process should be considered whenever mesenteric ischemia occurs in young patients, is noted at unusual sites, has a tendency to concomitantly involve both the small and large intestine, and is associated with genitourinary involvement. Knowledge of the systemic clinical manifestations in affected patients can suggest the presence of vasculitis and help establish the specific diagnosis.
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Footnotes
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Abbreviation: ANCA = antineutrophil cytoplasmic antibody
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References
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Abstract
Introduction
