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□ CASE REPORT □

Intestinal Involvement in Wegener’s GranulomatosisDiagnosed and Followed up by Double

Balloon Enteroscopy

Kazuko Beppu, Taro Osada, Kanako Inoue, Kenshi Matsumoto, Tomoyoshi Shibuya,

Naoto Sakamoto, Masato Kawabe, Akihito Nagahara, Tatsuo Ogihara and Sumio Watanabe

Abstract

Wegener’s granulomatosis (WG) is a multisystemic disease of unknown etiology characterized by necrotiz-

ing vasculitis and granulomatous inflammation (1-3). The disease typically involves the upper airways, lungs

and kidneys, and gastrointestinal involvement is uncommon. Described here is a 33-year-old man who pre-

sented at the hospital with abdominal pain. Colonoscopy revealed multiple ulcers, including round ulcers,

throughout the large intestine. Small bowel ulcers were detected by double balloon enteroscopy (DBE). Fur-

ther study confirmed that these ulcers were caused by gastrointestinal complications of WG. The patient was

administered prednisolone and cyclophosphamide and remains in remission. This case indicates the impor-

tance of considering a gastrointestinal complication of WG as the potential cause of abdominal symptoms

among WG patients as well as the use of DBE in detecting such a complication.

Key words: Wegener’s granulomatosis, double balloon enteroscopy, small intestinal ulceration

(Intern Med 50: 219-222, 2011)(DOI: 10.2169/internalmedicine.50.4188)

Introduction

Wegener’s granulomatosis (WG) is a multisystemic ne-

crotizing vasculitis of unknown etiology with distinct clini-

cal and histological features (1-3). Histologically, it consists

of necrotizing vasculitis, affecting mainly small and

medium-sized arteries (4). The disease typically involves the

upper airways, lungs and kidneys. Gastrointestinal involve-

ment is very rare and has been previously detected almost

entirely by autopsy studies.

Double balloon enteroscopy (DBE) enables examination

of the entire small intestine and simultaneous tissue sam-

pling, along with a variety of therapeutic interven-

tions (5, 6). We present here the first reported case of intes-

tinal involvement in WG that was detected and followed up

by DBE.

Case Report

The patient was a 33-year-old Japanese man who was di-

agnosed in 2007 with WG at the age of 32 when he pre-

sented with recurrent upper respiratory tract infections, he-

maturia, polyarticular joint pain, nasal bleeding and sus-

tained low-grade fever. This diagnosis was made according

to the criteria of the American College of Rheumatology

(ACR) (4) based on radiological evidence of pulmonary

cavities and the presence of granulomatous inflammation

with giant cells on nasal biopsies, in addition to positivity

for antineutrophil cytoplasmic antibody (C-ANCA). At that

time, he was administered oral medication (60 mg/day pred-

nisolone), which alleviated his symptoms.

In 2008, one year after the diagnosis, the patient, who

had been receiving treatment with prednisolone 1 mg/kg,

was admitted to the hospital due to abdominal pain. Physi-

cal examination revealed a body temperature of 38.8℃.

Blood pressure and pulse rate were 148/90 mmHg and 116

Department of Gastroenterology, Juntendo University, School of Medicine, Japan

Received for publication July 8, 2010; Accepted for publication October 6, 2010

Correspondence to Dr. Kazuko Beppu, kazukobp@juntendo.ac.jp

Intern Med 50: 219-222, 2011 DOI: 10.2169/internalmedicine.50.4188

220

Figure　1.　Colonoscopic examination shows round ulcers in the cecum.

Table　1.　Laboratory Findings on Admission

Peripheral bloodWBC 23000 /LRBC 3.54×1012 /LHb 9.3 g/dLHct 29.1 % Plt 295×109 /L

Blood chemistryTP 4.9 g/dLAlb 1.8 g/dLNa 135 mM/LK 4.4 mM/LCl 96 mM/LGlu106 mg/dLFe 10 μg/dLFerritin 2234 mg/mL

C3 124 mg/dLC4 15 mg/dLCH50 50 URF 310 IU/mLANA 20 ×anti-DNA (－)C-ANCA (－)P-ANCA (－)C7-HARP (－)

UrinalysisPH 8.0Glucose 1.2 g/dayProtein 1.2 g/dayOccult blood 1(+)

TIBC 188 μg/dLHbA1c 5.4 %CRP 11.8 mg/dLALP 578 IU/LAST 23 IU/LALT 58 IU/LLDH 275 IU/Lγ-GTP 100 IU/LT-Bil 1.07 mg/dLBUN 21 mg/dLCr 0.45 mg/dL

SerologyIgG 746 mg/dLIgA 176 mg/dLIgM 110 mg/dL

per minute, respectively. Findings of chest and abdominal

radiographs were unremarkable. Laboratory evaluation on

admission revealed a white blood cell count of 23,000/mm3,

C-reactive protein of 11.8 mg/dL and hemoglobin of 9.3 g/

dL. Although creatinine levels were normal, very slight he-

maturia and proteinuria were observed. In addition, C-

ANCA, which had been positive previously, was negative

(Table 1).

Colonoscopy showed round ulcers in the transverse colon,

cecum and terminal ileum (Fig. 1). A double-contrast bar-

ium study of the small intestine was performed and revealed

a ragged and narrow ileal colon (Fig. 2A). On the basis of

these results, retrograde double balloon enteroscopy (DBE)

(Fujinon EN-450T5/20; Fujinon Corp., Saitama, Japan) was

performed, which identified round ulcers in the ileal colon

(Fig. 2B). Multiple biopsies were performed; lesional biop-

sies showed inflammatory cells and fibrosis to the lamina

propia. Angiography revealed that multiple areas of stenosis

and irregular walls of the anterior and inferior mesenteric ar-

teries were caused by vasculitis (Fig. 3). Based on these

data, the final diagnosis was established as gastrointestinal

complications associated with WG.

The patient was switched from an ordinary diet to total

parenteral nutrition. In addition, the dosage of prednisolone

was increased to 2 mg/kg/day and 1 mg/kg/day of cyclo-

phosphamide was administered. The abdominal pain imme-

diately resolved, and after two subsequent weeks of treat-

ment, white blood cell count and C-reactive protein levels

normalized. Retrograde DBE performed two months after

the start of treatment indicated scarring from the initially de-

tected multiple ulcers and no new ulcerations in the ileum,

colon or rectum (Fig. 4A). Angiography revealed improve-

ment in the stenosis and irregular walls of the superior and

inferior arteries that had been initially observed (Fig. 4B).

The patient then resumed an ordinary diet and was given

oral systemic steroidal therapy; subsequently, symptoms

have been under control and the patient remains in remis-

sion.

Discussion

First described by Friedrich Wegener in 1936 (7), WG is

a form of vasculitis that affects various organs, particularly

the lungs and kidneys. The incidence of WG is 10 cases per

million per year, and it mainly occurs among the middle

aged. This disease can be effectively treated by daily ad-

ministration of prednisolone and cyclophosphamide. More-

over, because WG causes end-organ damage, long-term im-

Intern Med 50: 219-222, 2011 DOI: 10.2169/internalmedicine.50.4188

221

Figure　2.　A double contrast barium study of the small intestine shows a ragged and narrow ileal colon (A) and DBE image shows round ulcers located in the ileum (B).

A B

Figure　3.　Angiography image of the superior mesenteric ar-tery showing diffuse stenosis and irregular walls of the branches.

munosuppression is required (8). It is accompanied by long

periods of remission and relapse; hence, it is a critical ill-

ness.

Although focal necrotizing arteriolitis of the intestine has

been detected by necropsy in 24% of the 56 WG cases for

whom data are available (9), reports of significant clinical

manifestations of intestinal involvement are rare. Between

1982 and 2009, only 5 cases with severe intestinal involve-

ment were detected by emergency surgery due to perfora-

tion (10-14). In addition, 2 cases were detected by colono-

scopy (15, 16). The affected regions were in the terminal il-

eum, cecum and rectum in one of these patients and in the

terminal ileum and ascending colon in the other patient. En-

doscopic intestinal findings in these patients included small

ulcers and multiple ulcerations with bleeding and elevated

lesions. With regard to the present case, multiple round ul-

cers in the ileum could be detected by DBE in addition to

the colonic involvement found by colonoscopy.

The cause of ulcer formation in WG is generally consid-

ered to be vasculitis that obstructs blood flow to the intes-

tine. However, histological confirmation through examina-

tion of biopsy specimens obtained by endoscopy is not al-

ways possible, as biopsy specimens are generally too small

for a proper examination. This was true in the case reported

here. Although there was no detectable histological evidence

of vasculitis, intestinal involvement could be diagnosed by

ruling out other potential causes of small intestinal ulcers,

such as Crohn’s disease or tuberculosis, and on the basis of

the patient’s history, laboratory findings, including negative

fecal culture, and angiography results. As this case and pre-

vious reports suggest, endoscopic characteristics of intestinal

involvement in WG can be predicted by the presence of

multiple, small, round and clear ulcers associated with ob-

structed blood flow.

In summary, most significant is that this is the first re-

ported case of WG with intestinal involvement in which

DBE was used both to detect intestinal involvement and to

confirm non-recurrence. Also noteworthy is that although in-

testinal involvement is uncommon in WG, if left untreated,

it could be fatal. Therefore, when WG patients complain of

abdominal pain or exhibit abdominal symptoms without ap-

parent cause, intestinal complications due to vasculitis

should be strongly suspected and might be scrutinized using

DBE, the most optimal and up-to-date method for such a

purpose.

Intern Med 50: 219-222, 2011 DOI: 10.2169/internalmedicine.50.4188

222

Figure　4.　DBE examination revealing scarring from the initially detected multiple ulcers in the il-eum (A) and angiography image of the superior mesenteric artery shows multiple normal walls of the branches (B).

A B

The authors state that they have no Conflict of Interest (COI).

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