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Case 129: Polyarteritis Nodosa¹

¹ From the Department of Radiology, Dartmouth Hitchcock Medical Center, Lebanon, NH. Received January 8, 2004; revision requested March 9; revision received February 9, 2005; accepted March 2; final version accepted May 20.

Correspondence: Address correspondence to E.S.R., Baystate Franklin Medical Center, 164 High St, Greenfield, MA 01301 (e-mail: esrhodes{at}yahoo.com).

	HISTORY

TOP HISTORY IMAGING FINDINGS DISCUSSION References

 
A 68-year-old man presented with fever of unknown origin, generalized weakness, diarrhea, and abdominal pain. His symptoms had progressed for several weeks prior to admission. Initially, he experienced weight loss. This was followed by lower extremity edema that progressed to anasarca. The results of a comprehensive work-up for infection were negative and included negative blood and stool cultures and negative human immunodeficiency virus and Lyme disease test results. Results of transesophageal echocardiagraphy were negative for vegetations, and work-up was negative for hepatitis. Laboratory findings included a white blood cell count of 20 x 10⁹/L (normal level, [4 to 10] x 10⁹/L) and an albumin level of 1.2 g/dL (normal level, 3.2–5.2 g/dL). Liver function test results were normal. Antinuclear antibody test results were positive. This patient's erythrocyte sedimentation rate was 42 mm/h (normal level, 0–20 mm/h), and his C-reactive protein level reportedly was elevated when he had visited another hospital. Biopsy of the temporal artery was performed. Although the biopsy findings were difficult to interpret, the pathologist believed that severe giant cell temporal arteritis was the most appropriate diagnosis. Computed tomographic (CT) images of the sinuses and chest were normal, with the exception of bilateral pleural effusions. CT of the abdomen and pelvis was then performed to evaluate chronic abdominal pain and weight loss. This examination was followed by arteriography.

	IMAGING FINDINGS

TOP HISTORY IMAGING FINDINGS DISCUSSION References

 
CT (Fig 1) revealed pneumatosis intestinalis of the small bowel. Gas (likely portal venous gas) was seen within mesenteric vessels on multiple CT sections. In addition, there were air-fluid levels within the small bowel and small-bowel distention. Vasculature in the mesentery was dilated. Small nonspecific low-attenuating lesions were seen within both kidneys (Fig 1d). The frontal arteriogram of the superior mesenteric artery (Fig 2) showed multiple numerous small aneurysms.

View larger version (108K): [in this window] [in a new window] [Download PPT slide]   Figure 1a: Four noncontiguous transverse CT images (5-mm section thickness) of the abdomen and pelvis acquired with intravenous and oral contrast material. (a, b) Images show pneumatosis intestinalis (arrows) of the small bowel. Gas, likely portal venous gas (arrowheads), is also seen within mesenteric vessels. There are multiple distended loops and air-fluid levels in the small bowel. (c) Dilated vasculature in the mesentery (arrows) and gas (arrowhead) in a mesenteric vessel are visible. (d) Small low-attenuation lesions (arrows) can be seen within both kidneys.

View larger version (103K): [in this window] [in a new window] [Download PPT slide]   Figure 1b: Four noncontiguous transverse CT images (5-mm section thickness) of the abdomen and pelvis acquired with intravenous and oral contrast material. (a, b) Images show pneumatosis intestinalis (arrows) of the small bowel. Gas, likely portal venous gas (arrowheads), is also seen within mesenteric vessels. There are multiple distended loops and air-fluid levels in the small bowel. (c) Dilated vasculature in the mesentery (arrows) and gas (arrowhead) in a mesenteric vessel are visible. (d) Small low-attenuation lesions (arrows) can be seen within both kidneys.

View larger version (109K): [in this window] [in a new window] [Download PPT slide]   Figure 1c: Four noncontiguous transverse CT images (5-mm section thickness) of the abdomen and pelvis acquired with intravenous and oral contrast material. (a, b) Images show pneumatosis intestinalis (arrows) of the small bowel. Gas, likely portal venous gas (arrowheads), is also seen within mesenteric vessels. There are multiple distended loops and air-fluid levels in the small bowel. (c) Dilated vasculature in the mesentery (arrows) and gas (arrowhead) in a mesenteric vessel are visible. (d) Small low-attenuation lesions (arrows) can be seen within both kidneys.

View larger version (106K): [in this window] [in a new window] [Download PPT slide]   Figure 1d: Four noncontiguous transverse CT images (5-mm section thickness) of the abdomen and pelvis acquired with intravenous and oral contrast material. (a, b) Images show pneumatosis intestinalis (arrows) of the small bowel. Gas, likely portal venous gas (arrowheads), is also seen within mesenteric vessels. There are multiple distended loops and air-fluid levels in the small bowel. (c) Dilated vasculature in the mesentery (arrows) and gas (arrowhead) in a mesenteric vessel are visible. (d) Small low-attenuation lesions (arrows) can be seen within both kidneys.

View larger version (147K): [in this window] [in a new window] [Download PPT slide]   Figure 2: Frontal arteriogram of the superior mesenteric artery after contrast material injection shows multiple numerous small aneurysms (some noted with arrows) involving the mesenteric arterial branches.

	DISCUSSION

TOP HISTORY IMAGING FINDINGS DISCUSSION References

Vasculitides can cause focal or widespread pathologic changes in the abdo-men, including mesenteric ischemia, paralytic ileus, submucosal edema, hemorrhage, or bowel perforation (4). Pneumatosis resulting from vasculitis is presumed secondary to ischemia and results in mucosal disruption and bowel necrosis (1). There is no universally accepted classification, and there is overlap between individual entities. Vasculitides that involve the gastrointestinal tract include Takayasu arteritis, polyarteritis nodosa, Wegener granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, systemic lupus erythematosus, and rheumatoid vasculitis (4). Radiologic findings in the various types of vasculitis overlap considerably. Knowledge of the systemic clinical manifestations can help establish a specific diagnosis. Takayasu arteritis typically occurs in women and usually affects the aorta (5). Wegener granulomatosis affects the upper respiratory tract in more than 90% of cases and the kidneys in up to 80% of cases (6). This patient did not have a history of upper respiratory tract or sinus disease that would suggest a diagnosis of Wegener granulomatosis, and CT scans of the chest and sinuses were normal, with the exception of bilateral pleural effusions. Churg-Strauss syndrome shares features with Wegener granulomatosis and polyarteritis nodosa; however, it is distinguished by the presence of asthma, allergy symptoms, and peripheral and tissue eosinophelia (7). Diagnosis of systemic lupus erythematous and rheumatoid vasculitis is aided by distinct features in their clinical manifestations. Patients with systemic lupus erythematosus frequently present with symptoms of arthritis, photosensitivity, and cutaneous manifestations, including characteristic malar rash, discoid rash, and oral ulcers (4). These symptoms were not present in this patient. Rheumatoid vasculitis typically occurs in patients with advanced rheumatoid arthropathy. Microscopic polyangiitis is a small-vessel vasculitis and is not associated with aneurysms at arteriography (4). Scleroderma can manifest as a vasculitis. Connective tissue disorders typically involve segmental occlusion of vessels, secondary to antibody-antigen complex deposition, that results in endothelial damage, fibrinoid thickening, and intimal hyperplasia. This ultimately leads to an obliterative endarteritis of the digital vessels (8). The presence of aneurysms on the mesenteric angiogram, as well as the absence of the characteristic skin thickening, tightness, and induration, makes scleroderma an unlikely diagnosis. Polyarteritis nodosa frequently involves the gastrointestinal tract. The clinical features of polyarteritis nodosa include onset of constitutional symptoms over weeks or months. Patients often present with fever, malaise, weight loss, and myalgias. Gastrointestinal manifestations occur in approximately half of patients. Patients with renal involvement can have proteinuria or hematuria (9).

It is often difficult to diagnose polyarteritis nodosa because of overlapping clinical features among the various vasculitides. Angiography can be a useful tool if a patient is suspected of having this disease. In this patient, abdominal visceral arteriography was performed (Fig 2) because of concern for systemic vasculitis. This study revealed muliple aneurysms involving the superior mesenteric (Fig 2), renal (Fig 3a), and hepatic (Fig 3b) arteries. The findings of multiple small aneurysms on abdominal arteriograms have often been considered pathognomonic for polyarteritis nodosa. However, multiple aneurysms may be seen in numerous other disorders, including heroin and methamphetamine abuse, Wegener granulomatosis, systemic lupus erythematosus, diabetes, rheumatoid arthritis, and—rarely—giant cell temporal arteritis (10–12). In addition, a rare disorder, segmental mediolytic arteriopathy, has been reported to mimic polyarteritis nodosa in the splenic and hepatic arteries (13). Although the finding of multiple aneurysms is not pathognomonic for polyarteritis nodosa, Hekali et al (12) analyzed 156 patients in whom the indication for mesenteric angiography was suspicion of arteritis. In this group, the researchers found that angiography had a sensitivity of 89% and a specificity of 90% for polyarteritis nodosa (12). Without angiography, the correct diagnosis can be established with biopsy of subcutaneous nodules or skeletal muscle. However, the diagnostic success rate of biopsy has been disappointing, as sampling errors and lack of disease specificity limit the value of biopsy (14). The low yield of biopsy raises the diagnostic value of angiography in the diagnosis of polyarterits nodosa.

View larger version (170K): [in this window] [in a new window] [Download PPT slide]   Figure 3a: Frontal (a) renal and (b) hepatic arteriograms. There are multiple numerous small aneurysms (some noted with arrows) involving the renal and hepatic arteries. In a, decreased perfusion to the lower pole, consistent with a renal infarct, is visible.

View larger version (173K): [in this window] [in a new window] [Download PPT slide]   Figure 3b: Frontal (a) renal and (b) hepatic arteriograms. There are multiple numerous small aneurysms (some noted with arrows) involving the renal and hepatic arteries. In a, decreased perfusion to the lower pole, consistent with a renal infarct, is visible.

Polyarteritis nodosa is a systemic necrotizing vasculitis of medium-sized arteries (9). The characteristic pathologic findings are fibrinoid necrotizing inflammatory foci in the walls of small- and medium-sized arteries, with multiple small aneurysms. In the acute phase, the elastic lamina is destroyed, and the media undergoes fibrinoid necrosis. Microaneurysms arise from these weakened portions of the wall. In the reparative and chronic stages, fibroblast proliferation causes wall thickening and may produce areas of stenosis or occlusion. These findings are best seen with digital subtraction arteriography, although CT can depict dilated mesenteric vessels and larger aneurysms. Stenosis or occlusion may lead to ischemia or infarction (5). The kidney is most commonly involved, followed by the heart, gastrointestinal tract, liver, spleen, and pancreas (18). If untreated, polyarteritis nodosa is usually fatal. However, treatment with corticosteroids and cyclophosphamide improves the chance for survival.

Polyarteritis nodosa occurs twice as often in male than female patients and typically occurs in the 5th through 7th decades of life (19). Symptoms are variable and include fever, malaise, and abdominal pain. Other clinical features may include myalgias, arthralgias, hypertension, and mononeuritis multiplex. Leukocytosis with elevation of hepatic transaminase and alkaline phosphatase levels may be noted. An association with hepatitis B surface antigen seropositivity has been documented (5). Renal polyarteritis nodosa may manifest as acute or chronic renal failure, nephritic syndrome, or perirenal hemorrhage. Gastrointestinal polyarteritis nodosa may produce ulceration, perforation, hemorrhage, or infarction and the corresponding symptoms.

In this patient with pneumatosis intestinalis and portal venous gas, the clinical history, laboratory findings, and temporal artery biopsy results should have raised concern for a systemic vasculitis. Among the vasculitides involving the abdomen, polyarteritis nodosa was the best fit given the patient's clinical history and the distribution of disease. Biopsy findings suggesting giant cell temporal arteritis should not preclude the diagnosis of polyarteritis nodosa. This case reinforces that differentiating between vasculitides and arriving at a correct diagnosis are difficult and require integration of clinical, radiologic, and pathologic findings. In light of the arteriographic findings, a final diagnosis of polyarteritis nodosa was rendered by the pathologist, despite the initial interpretation of pathologic findings favoring a diagnosis of giant cell temporal arteritis. This case emphasizes the importance of arteriography as a diagnostic tool in the evaluation of vasculitis.

	FOOTNOTES

 
Authors stated no financial relationship to disclose.

Part one of this case appeared 4 months previously and may contain larger images.

 

	References

TOP HISTORY IMAGING FINDINGS DISCUSSION References

 

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