AJR 2000; 174:1675-1679
© American Roentgen Ray Society
Radiologic Findings of Abdominal Polyarteritis Nodosa
Keum Nahn Jee1,
Hyun Kwon Ha1,
In Jae Lee1,
Jeong Kon Kim1,
Kyu-Bo Sung1,
Kyoung-Sik Cho1,
Pyo Nyun Kim1,
Moon-Gyu Lee1,
Hyo Keun Lim2,
Cheol-Soon Choi3 and
Yong Ho Auh1
1
Department of Radiology, Asan Medical Center, University of Ulsan College of
Medicine, 388-1 Poongnap-Dong, Songpa-Ku, Seoul, 138-736, Korea.
2
Department of Radiology, Sam-Sung Medical Center, Sungkyunkwan University
College of Medicine, 50 Irwon-Dong, Kangnam-Ku, Seoul, 135-710, Korea.
3
Department of Radiology, Kang Dong Sacred Heart Hospital, Hallym University
College of Medicine, 445 Gil-Dong, Kangdong-Ku, Seoul, 134-701, Korea.
Received September 2, 1999;
accepted after revision November 8, 1999.
Presented at the annual meeting of the American Roentgen Ray Society, New
Orleans, May 1999.
Address correspondence to H. K. Ha.
Abstract
OBJECTIVE. The purpose of this study was to evaluate the
radiographic and imaging findings of seven patients with polyarteritis nodosa
involving the abdomen.
CONCLUSION. Vasculitis should be considered when multiple abdominal
organs have abnormal radiologic findings; however, angiography is necessary to
specifically diagnose polyarteritis nodosa.
Introduction
Polyarteritis nodosa is a progressive inflammatory vasculitis,
characterized by progressive fibrinoid necrotizing inflammatory foci in the
walls of small- and medium-sized muscular arteries; a characteristic finding
is the presence of aneurysms as large as 1 cm in diameter. The incidence is
unknown, but this uncommon disease affects people in their fourth and fifth
decades of life; the male-to-female ratio is 2-3:1. The primary abnormal
changes of the involved vessel consist of fibrinoid or hyaline necrosis of the
media with simultaneous or subsequent involvement of the intima and
adventitia. Secondary changes include aneurysm formation, hemorrhage, and
thrombosis. The kidney is most commonly involved (80-100%), followed by the
heart (up to 70%), gastrointestinal tract (50-70%), liver (50-60%), spleen
(45%), and pancreas (25-35%)
[1]. If untreated,
polyarteritis nodosa is usually fatal as a result of progressive renal failure
or gastrointestinal complications. However, therapy with corticosteroids and
cyclophosphamide results in remission or cure in 90% of patients
[2].
Until recently, little had been reported in radiology literature regarding
polyarteritis nodosa [3]. To
our knowledge, most of the reports have focused on angiography. The purpose of
this study was to evaluate the radiographic and imaging findings of seven
patients with polyarteritis nodosa involving the abdomen.
Materials and Methods
We reviewed the medical records and radiologic findings of seven patients
with polyarteritis nodosa involving the abdomen; these cases were collected
from three institutions. The patients, three men and four women, were 23-58
years old (mean, 47 years). The diagnosis was made by angiographic findings
(n = 6) findings and pathologic reports obtained from the surgical
(n = 2) or biopsy (n = 5) specimens of the bowel, muscle, or
skin. The patients had symptoms and signs of diffuse abdominal pain
(n = 6), hypertension (n = 4), abdominal bruit (n =
1), hematochezia (n = 2), fever and chills (n = 3), skin
rash (n = 1), and right-sided flank pain with gross hematuria
(n = 1), with a duration of several days to more than four years.
Positive laboratory findings included elevation of erythrocyte sedimentation
rate (n = 7), leukocytosis (n = 5), and positive rheumatoid
factor (n = 2). There was no positive hepatitis B—virus
antigenemia in six patients tested. Of the three patients who underwent
serologic tests for antineutrophilic cytoplasmic autoantibody, two had
positive results. The radiologic studies available in these seven patients
were angiography in seven, abdominal CT in four, MR imaging in two, and
small-bowel follow-through examination in three. Surgery (segmental bowel
resection) was performed in two of the seven patients. The indications for
surgery were persisting hematochezia with rapidly decreasing hemoglobin level
in one patient and clinical symptoms and signs of bowel obstruction in the
other.
CT scans were performed using a 9800 Quick System (General Electric Medical
Systems, Milwaukee, WI) and Somatom Plus-S, Plus-4, and Plus-40 (Siemens,
Erlangen, Germany) with 8- or 10-mm slice thickness at 8- or 10-mm intervals
from the dome of the liver to the symphysis pubis. Approximately 600-900 ml of
oral contrast material ([2% barium sulfate suspension] E-Z CAT; E-Z-EM,
Westbury, NY) was administered 1 hr before scanning in all patients except one
who was suspected of having small-bowel obstruction. One hundred to one
hundred twenty milliliters of IV contrast material ([iopamidol] Iopamiro 300,
Bracco, Milan, Italy; or [iopromide] Ultravist, Schering, Berlin, Germany) was
administered as a bolus at 3 ml/sec. Scanning was started 40-60 sec after IV
infusion with a scanning time of 0.8-2.0 sec and an interscan delay of 1.8-3.5
sec.
MR imaging was performed in two patients because of the presence of
residual barium in the bowel from a previous small-bowel follow-through
examination in one and a physician's request in the other. MR imaging was
obtained with a 1.5-T superconductive unit (Magnetom Vision; Siemens) using a
body phased array coil. The MR imaging sequences used were breath-hold coronal
and axial T1-weighted fast low-angle shot two-dimensional imaging and
T2-weighted half-Fourier acquisition single-shot turbo spin-echo imaging.
We evaluated involvement patterns in the abdominal organs and radiographic
and imaging features in these seven patients. The findings were
retrospectively analyzed by two radiologists. If their interpretations
differed, a consensus was reached for the final decision.
Results
Radiographic, CT, and MR imaging findings are summarized in
Table 1.
Six of the seven patients had involvement of the gastrointestinal tract
(Figs.
1A,1B,1C,1D,2A,2B,3).
Mesenteric artery involvement was confirmed at angiography in five of the six
patients. In the sixth patient, involvement was confirmed on histopathologic
examination after segmental bowel resection for small-bowel obstruction. On
angiography, multiple microaneurysms (Fig.
1B) were seen at the jejunal, ileal, right-colic, and midcolic
branches of the superior mesenteric artery. In one patient, the inferior
mesenteric artery branches were also involved. Other angiographic findings
included vascular narrowing and irregularity in three patients and focal areas
of hypervascularity in one. Of the six patients with involvement of the
gastrointestinal tract, two underwent CT, one underwent MR imaging, and one
underwent both CT and MR imaging. CT and MR imaging showed bowel wall
thickening in three patients (small intestine in two patients and small
intestine, colon, and rectum in one patient), with target sign in the
thickened bowel wall in two, mesenteric vascular engorgement and haziness in
three, and ascites in two. In one patient, MR imaging showed high-grade
small-bowel obstruction with beaklike luminal narrowing at the distal jejunum
(Fig.
2A,2B).
In the other patient, in whom neither CT nor MR imaging was performed,
small-bowel follow-through examination showed diffuse mucosal fold thickening
from the duodenum through the ileum (Fig.
3).
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Fig. 1A. —42-year-old man with fever, myalgia, arthralgia, and peripheral
neuropathy. Contrast-enhanced CT scan shows diffuse bowel wall thickening of
small (asterisks) and large (arrowheads) intestine with
target sign. Note bilateral ureteral dilatation (arrows) and large
amount of ascites.
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Fig. 1B. —42-year-old man with fever, myalgia, arthralgia, and peripheral
neuropathy. Superior mesenteric arteriogram shows multiple microaneurysms
(arrows) in peripheral branches of superior mesenteric artery.
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Fig. 1D. —42-year-old man with fever, myalgia, arthralgia, and peripheral
neuropathy. Follow-up contrast-enhanced CT scan obtained 1 month after
A, after treatment with steroid therapy, shows improvement of bowel
wall thickening. However, both bilateral ureteral dilatation (arrows)
and ascites are still visible.
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Fig. 2A. —55-year-old woman with diffuse abdominal pain and vomiting for 5
days. Overhead radiograph in small-bowel follow-through examination shows
abrupt, concentrical luminal narrowing (arrows) of jejunum with
high-grade small-bowel obstruction, simulating jejunal malignancy.
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Fig. 2B. —55-year-old woman with diffuse abdominal pain and vomiting for 5
days. Coronal half-Fourier acquisition single-shot turbo spin-echo MR image
(TR/TE, 4/59; matrix, 128 x 256; field of view, 338 x 450; flip
angle, 140°; slice thickness, 7 mm; and no interslice gap) shows short
length of luminal narrowing (arrowheads) of jejunum and diffuse
dilatation of proximal intestinal loop. Note residual barium suspension
(asterisks) in small-intestine loop, which appears to be
hypointense.
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Fig. 3. —58-year-old man with abdominal pain and hematochezia. Upper
gastrointestinal radiography shows diffuse fold thickening of duodenum.
Similar findings were seen in small-intestine loop (not shown).
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The genitourinary system was involved in five of the seven patients (Figs.
1A,1B,1C,1D,
4A,4B,4C,
and 5); the unilateral renal
artery in one; bilateral renal arteries in two; bilateral renal arteries and
middle one third of the ureter in one; and bilateral renal arteries, bilateral
ureterovesical junction of the ureter, and bladder in one. Renal artery
involvement was confirmed on angiography on which multiple aneurysms (Fig.
4A,4B,4C)
were noted in the arcuate and interlobar branches of the renal artery in these
five patients and slight luminal irregularities of the arterial wall in two of
the five. In these five patients, CT or MR imaging was available in four
patients and showed multifocal areas of renal infarction in two, bilateral
ureteral obstruction in two, perinephric hematoma
(Fig. 5) caused by aneurysmal
rupture in one, and diffuse bladder wall thickening in one.
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Fig. 5. —42-year old man with abrupt right-sided flank pain and gross
hematuria. Contrast-enhanced CT scan shows right perinephric hematoma (H).
Note multifocal areas of tiny parenchymal defects (arrows) in spleen
caused by infarction.
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The liver was involved in four of the seven patients, with multiple
aneurysms (n = 4) (Fig.
1C) in the hepatic arterial branches on angiography and focal
parenchymal infarction (n = 1) on CT. The spleen was involved in
three patients; multiple aneurysms were revealed on angiography in all three
of these patients, and infarctions (Fig.
5) were noted in two of the three. In one patient, left gastric
artery branches were seen to be involved with multiple aneurysms on
angiography.
Discussion
The exact cause of polyarteritis nodosa is not clear. In the past, some
investigators believed that drugs and serum therapy, including sulfonamides,
serums, vaccines phenytoin sodium, iodine, thiouracil, and many other drugs,
were possible causative agents
[4]. However, other conditions,
such as bacterial infection, toxic injury, diseases of the central nervous
system, and viral infection, were also proposed as possible contributing
agents [4]. Therefore, it is
likely that polyarteritis nodosa creates a nonspecific hypersensitivity to
numerous antigens. The disease process chiefly involves the small- and
medium-sized arteries and arterioles in a segmental fashion; the venules are
rarely involved. Although none of our patients had positive hepatitis
B—virus surface antigenemia, 36% of patients with polyarteritis nodosa
have been reported to have hepatitis B antigen in their serum
[1].
The clinical diagnosis of polyarteritis nodosa is usually made on the basis
of various symptoms and signs of abdominal pain, hypertension, arthralgia or
arthritis, peripheral and central nervous system dysfunction, retinopathy, and
cardiac, respiratory, and hepatic involvement
[4]. Such protean clinical
manifestations frequently present a diagnostic challenge. Without angiography,
the correct diagnosis is established only by obtaining a biopsy of
subcutaneous nodules or skeletal muscle; however, the diagnostic success rate
of the biopsy has been disappointing (20-35%)
[5]. Therefore, a negative
biopsy does not rule out the condition. In addition, antineutrophilic
cytoplasmic autoantibodies are antibodies directed against certain proteins in
the cytoplasm of neutrophils and are often present in the serum of patients
with polyarteritis nodosa and correlate with their disease activity. However,
this test is not specific for cases of polyarteritis nodosa; it is also
positive in Churg-Strauss syndrome, Wegener's granulomatosis, and microscopic
polyangitis [6]. Many authors
have agreed that angiography helps to confirm the suspected clinical
diagnosis. Findings of multiple, small-sized aneurysms on angiography are
pathognomonic for the disease. The aneuryms are usually multiple (most often
10 or more in any one visceral circulation) and 2-5 mm, commonly affecting the
branch points of arteries. Studies regarding the frequency of this finding
have been limited. According to a study by Travers et al.
[1], angiography revealed
aneurysms in 60-80% of patients.
The kidney is the most frequently affected organ. Depending on the vessels
involved, renal polyarteritis nodosa may present clinically as acute or
chronic renal failure or nephrotic syndrome or may be manifested by perirenal
hemorrhage. With renal complications, hypertension often develops rapidly. Two
characteristic lesions have been described in polyarteritis nodosa when the
kidney is involved: an arteritis and a glomerulitis that may occur separately
or together [7]. The arteritis
primarily involves the arcuate vessels and consists of fibrinoid necrosis and
vascular thrombosis. Thus, multiple small renal infarcts are a prominent
feature. The aneurysm, forming as a result of focal necrosis of the vessel
wall, may undergo necrosis and healing with fibrosis, but occasionally will
rupture and produce perirenal and retroperitoneal hemorrhage
[7], as occurred in one of our
cases. Two of our patients had involvement of the extrarenal genitourinary
system (ureter and bladder). Hydronephrosis can occur in patients with
polyarteritis nodosa and in patients with other types of vasculitis such as
systemic lupus erythematosus and
Henoch-Schönlein syndrome and seems to be caused
by either detrusor muscle spasm with subsequent vesicoureteral reflux or
fibrosis of the ureterovesical junction
[8].
Approximately one half to two thirds of patients with polyarteritis nodosa
have abdominal pain, nausea, vomiting, or other gastrointestinal symptoms. The
gastrointestinal lesions may take the form of ulceration, perforation,
hemorrhage, or infarct and produce corresponding symptoms: gastrointestinal
hemorrhage occurs in roughly 6% of cases; bowel perforation in 5%; and bowel
infarction in 1% [1,
2]. In the gastrointestinal
tract, the jejunum appears to be most commonly involved. Generally, when the
aneurysms involve the mesenteric arteries, their number and size are fewer and
smaller compared with those of the kidney and liver
[9]. The infarcted bowel
progresses into either bowel perforation or bowel obstruction caused by
stricture. As in one of our cases with focal bowel stricture caused by
mesenteric ischemia, differentiation from malignant or chronic granulomatous
inflammatory bowel diseases may be difficult on barium or imaging studies.
In addition to the genitourinary and gastrointestinal tracts, any organ in
the abdomen can be involved. The liver is the most common site of involvement
in patients with polyarteritis nodosa. The vascular changes in the liver
result in aneurysm formation, rupture of aneurysm, infarction, interstitial
hepatitis, and liver cirrhosis. Although the liver is commonly involved, there
are few clinical signs or symptoms suggesting hepatic involvement
[9]. The cystic arteries are
also a frequent site of developing arteritis, and subsequent acalculous
cholecystitis and intracholecystic hemorrhage have been reported
[10]. According to a series
describing 17 patients with polyarteritis nodosa
[11], the pancreas was
involved in eight cases and the spleen in three cases.
Because a definite diagnosis depends largely on the detection of
small-sized aneurysms on angiography, the specificity of this finding should
be taken into consideration. Drug abuse is an accepted cause of multiple
microaneurysms in various organs. Citron et al.
[12] reported that the diverse
clinicopathologic syndrome and angiographic findings of necrotizing angiitis
associated with drug abuse were strikingly similar to those of polyarteritis
nodosa with severe renal, gastrointestinal, cardiac, and neurologic
involvement. Other vasculitides, such as Wegener's granulomatosis and systemic
lupus erythematosus, have also been reported in the literature to have
multiple aneurysms similar to those of polyarteritis nodosa
[13].
In conclusion, the possibility of vasculitis should be considered when
multiple abdominal organs have abnormal radiologic findings; however,
angiography is necessary for establishing a specific diagnosis of
polyarteritis nodosa.
Acknowledgments
We thank Bonnie Hami, Department of Radiology, University Hospitals Health
System, Cleveland, OH, for editorial assistance in preparing this
manuscript.
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Abstract
Introduction
