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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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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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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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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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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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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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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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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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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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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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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Copyright © 2000 by the Radiological Society of North America.
