DOI:10.2214/AJR.05.1596
AJR 2006; 187:W396-W398
© American Roentgen Ray Society
Radiologic Findings of Gastrointestinal Complications in an Adult Patient with Henoch-Schönlein Purpura
Dong Jin Chung1,
Yong Sung Park1,
Kyu Chan Huh2 and
Ji Hyung Kim1
1 Department of Radiology, University of Konyang School of Medicine, 685
Gasuwon-dong, Seo-gu, Daejeon, South Korea 302-718.
2 Department of Gastroenterology, University of Konyang College of Medicine,
Daejeon 302-718, South Korea.
Received September 8, 2005;
accepted after revision October 20, 2005.
Address correspondence to D. J. Chung
(bookdoo7{at}chollian.net).
WEB This is a Web exclusive article.
Keywords: colonoscopy • CT • gastrointestinal radiology • Henoch-Schönlein purpura • small bowel
Introduction
Henoch-Schönlein purpura (HSP) is a systemic, generalized vasculitis
of small vessels of the skin, joint, gastrointestinal tract, and kidney. Its
cause is thought to be an IgA-containing immune complex-mediated autoimmune
disease. HSP was originally described in children, but it can also affect
adults [1]. It is frequently
associated with gastrointestinal involvement, but perforation and stricture
are rare because large vessels are unaffected and tend to recover
spontaneously and heal without sequelae. To our knowledge, the radiologic
findings of gastrointestinal complications in adult HSP have not been reported
previously. Herein, we present the radiologic findings of a 54-year-old man
who had HSP with small-bowel perforation and stricture. Histopathologic and
endoscopic findings were reviewed to diagnose HSP.
Case Report
A 54-year-old man was admitted to the hospital with severe epigastric pain
and several episodes of vomiting over a 2-day period. On physical examination,
he was stuporous and had unstable vital signs. His pulse was 130 beats per
minute; blood pressure, 70/40 mm Hg; respiration rate, 44 breaths per minute;
and temperature, 38.5°C. His oxygen saturation was 65% while breathing
100% oxygen with the use of a face mask. His trachea was intubated, and he was
placed on ventilator support. At presentation, no rash was evident. He had a
history of diabetes mellitus but had not taken any hypoglycemic agents. He
denied hematemesis, melena, or hematochezia. Initial laboratory studies
revealed negative fecal occult blood; proteinuria; hematuria; WBC, 5.25
x 103/µL; hemoglobin, 10.2 g/dL; platelet count, 360
x 103/µL; C-reactive protein, 7.1 mg/dL (normal < 0.2);
glucose, 274 mg/dL; blood urea nitrogen and creatinine, 39 and 3.35 mg/dL; and
albumin, 2.4 g/dL.
Contrast-enhanced abdominopelvic CT of the patient showed circumferential
mural thickening of the small bowel, seen from the proximal jejunum to the
terminal ileum, and mesenteric haziness (Figs.
1A,
1B,
1C,
1D, and
1E). At the distal ileum, the
bowel wall was focally defective, which was sufficient to make a diagnosis of
bowel perforation. A small-bowel follow-through revealed a short stenotic
segment in the distal ileum with fold thickening and a partly widened proximal
lumen, suggesting a low-grade, incomplete small-bowel obstruction. Subsequent
colonoscopy was performed into the distal ileum, which was remarkable for
erythematous mucosa, linear ulcers, and stricture. The obstructed segment
prevented colonoscopic passage. The rectum also showed erythema and aphthous
ulcers without stricture.
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Fig. 1A —54-year-old man with Henoch-Schönlein purpura involving
small bowel. Axial contrast-enhanced CT scan shows diffuse small-bowel wall
thickening (arrows) and increased mesenteric fat density
(arrowhead).
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Fig. 1B —54-year-old man with Henoch-Schönlein purpura involving
small bowel. Axial contrast-enhanced CT scan obtained several centimeters
caudal to A shows focal wall defect area, suggesting perforation at
distal ileum with wall thickening (arrow). Extraluminal gas is
absent.
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Fig. 1C —54-year-old man with Henoch-Schönlein purpura involving
small bowel. Small-bowel follow-through reveals short stenotic segment of
distal ileum with fold thickening (arrow) and partly widened proximal
lumen (arrowhead).
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Fig. 1D —54-year-old man with Henoch-Schönlein purpura involving
small bowel. Colonoscopy shows erythematous mucosa (arrow) and linear
ulcers (arrowheads). Colonoscopic passage through obstructed segment
was not possible.
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Fig. 1E — 54-year-old man with Henoch-Schönlein purpura involving
small bowel. Microscopic finding shows acute necrotizing changes in
leukocytoclastic vasculitis (vessel and gland destruction by inflammatory cell
infiltration) (arrows).
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Twenty-two days after admission, a palpable cutaneous purpura developed on
the patient's leg. Histologic findings of the terminal ileum and skin were
consistent with necrotizing changes of leukocytoclastic vasculitis in mucosal
small vessels, with the presence of fibrinoid necrosis. Immunofluorescent
kidney biopsy findings revealed moderate IgA and C3 deposits in the mesangium
and along peripheral capillary loops.
Based on the clinical picture and histologic findings, a diagnosis of HSP
was made. The patient was started on IV corticosteroids (prednisolone, 60
mg/d), and symptoms of abdominal pain resolved, which was followed by an
improvement of the purpura. Two days after treatment, he had complete relief
of abdominal pain and vomiting, but proteinuria increased and renal
insufficiency continued. He did not undergo laparotomy or surgical resection
of the stenotic segment of the distal ileum. A follow-up colonoscopy after 2
weeks of treatment showed marked resolution of the erythema and ulcers in the
distal ileum and slight improvement of the stenotic lesion.
Discussion
HSP affects children and young to middle-aged adults. The disease occurs
most commonly in children between 3 and 10 years old; however, in some series,
30% of patients have been reported to be older than 20 years
[2]. The clinical presentation
may be a triad of palpable purpura, arthritis, and abdominal pain. Cream et
al. [3] reported a series of 77
adult cases of HSP in which 44% had gastrointestinal symptoms. Interestingly,
gastrointestinal manifestations precede cutaneous lesions in 10-15% of
patients [4], as occurred in
our case. This fact may be of great clinical significance, because these
gastrointestinal manifestations are usually serious and can result in invasive
diagnostic techniques, including laparotomy. In adults, a diagnosis of HSP
requires the presence of at least two of the flowing four criteria: palpable
purpura, age younger than 20 years when symptoms first appear, bowel angina,
and biopsy with granulocytes in vascular walls. The presence of two or more of
these criteria identifies the illness with a sensitivity of 87.1% and a
specificity of 87.7% [5]. Our
patient presented with three of these criteria.
CT findings of the gastrointestinal manifestations of HSP included a
thickened bowel, free peritoneal fluid, ileus of affected loops, and bowel
dilatation. Jeong et al. [6]
suggested that HSP should be considered when CT scans show multifocal areas of
bowel wall thickening, mesenteric edema, vascular engorgement, and nonspecific
lymphadenopathy. They suggested that the CT findings of a vascular disorder of
the small intestine are bowel wall thickening of less than 1.2 cm at its
greatest dimension and ancillary findings of mesenteric changes, such as
vascular engorgement and mesenteric edema.
In adults the disease process is identical to that in children, and the
course of the illness is also similar. However, gastrointestinal involvement
is less common in adults, and bowel perforation and obstruction are rare.
Because full-thickness necrosis is rare, patients with HSP tend to recover
spontaneously and heal without sequelae, although scarring and stenosis may
develop in some cases. After being treated with corticosteroids,
gastrointestinal lesions in HSP are usually reversible and heal, although a
few patients (2-6%) with HSP develop conditions requiring surgery.
Vasculitis comprises a broad group of syndromes characterized by
inflammation and necrosis in the walls of blood vessels, which result in
perforation. Vasculitis and subsequent thrombosis can cause ischemic change of
the bowel wall, and intestinal infarction can also sometimes occur. Shirahama
et al. [7] described the
usefulness of sonography for detecting intestinal complications. They found
that ischemia is indicated when an arterial signal or color Doppler flow
cannot be detected. Chronic intestinal obstruction after HSP has been reported
in some patients [8], and it
was presumed that this occurred secondary to ischemic insult and focal
ulceration.
In conclusion, although HSP does not generally cause late complications in
the gastrointestinal tract, the present case indicates that bowel perforation
and stricture can develop secondary to an acute vasculitic ischemic
insult.
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Introduction
Case Report
