LETTER TO THE EDITOR

Intestinal perforation as the initial presentationof Wegener’s granulomatosis

Hakan Bulus • Erdem Kocak • Alper Yavuz •

Muzaffer Akkoca • Ali coskun • Seyfettin Koklu

Received: 3 March 2012 / Accepted: 23 August 2012

� Springer-Verlag 2012

To the Editor

Wegener’s granulomatosis is a necrotizing vasculitis of

unknown etiology characterized mainly by inflammation of

the small- and medium-sized arteries and veins that affect

any viscera [1]. Gastrointestinal involvement is rather

uncommon and is presented with mild-to-severe life-

threatening complications. We report a female patient with

WG who presented with massive intestinal perforation as

the initial presentation.

A 47-year-old female patient was admitted to our hos-

pital with the complaints of abdominal pain, nausea, and

vomiting. On physical examination, blood tension was

100/60 mmHg; pulse rate, 115/min; and the temperature,

39 �C. Abdominal examination revealed diffuse rebound

tenderness and diminished bowel sounds. Her detailed

history revealed that she had been admitted to another

hospital because of recurrent sinusitis two weeks ago. She

had been treated with antibiotics and nasal decongestants.

Initial laboratory findings were as follows: serum hemo-

globin, 8.1 g/dl; white cell count, 21,000/mm3; and platelet

count, 125,000/mm3; erythrocyte sedimentation rate,

68 mm/h; and positive anti-neutrophil cytoplasmic anti-

body (c-ANCA). Her liver and renal functional tests were

normal. Chest X-ray showed multiple round opacities in

the right lung, and the plain abdominal radiograph revealed

subdiaphragmatic free air. Contrast-enhanced computer-

ized tomography scan showed small bowel thickening with

intra-abdominal free air and fluid. The patient was operated

in the emergent condition with these findings. During

surgery, multiple ischemic, necrotic, and perforated areas

in the jejunal and ileal regions were detected. After intra-

abdominal irrigation, massive small intestinal resection and

jejunostomi were performed. The surgically resected small

intestine measured 130 cm in length. The mucosal surface

of the resected specimen showed multiple transversely

oriented shallow ulcers (Fig. 1). Histopathological exami-

nation showed inflammatory infiltration associated with

necrotizing and granulomatous vasculitis of small-to-

medium-sized vessels, which was consistent with WG. The

day after surgery, oral cyclophosphamide and prednisolone

treatment was started with the diagnosis of WG. On the

third post-operative day, severe dyspnea and anuria were

H. Bulus � A. Yavuz � M. Akkoca � A. coskun

Department of General Surgery, Kecioren Training and Research

Hospital, Ankara, Turkey

E. Kocak (&)

Department of Gastroenterology, Siirt State Hospital,

Siirt, Turkey

e-mail: kocak67@hotmail.com

S. Koklu

Department of Gastroenterology, Hacettepe University,

Ankara, Turkey

Fig. 1 The resected specimen showed multiple transversely oriented

shallow ulcers

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DOI 10.1007/s00296-012-2517-y

developed and the patient was died due to multiorgan

failure.

Essentially, all patients with WG present with upper

airway or pulmonary involvement, and the majority have

both. Because of its variable presentation, there is often a

delay in the diagnosis. It can rapidly lead to death in people

with multiple affected organs, who do not get appropriate

treatment. During the course of WG, intestinal involvement

is uncommon and usually detected in autopsy studies [2].

Reviewing the literature, only few cases have been reported

with intestinal perforation associated with WG [3–5]. The

ileal perforation is seen more commonly in men, usually

manifest in age between age 26 to 56, and occur within the

first year of the disease [6].

The present case had been admitted to another hospital

two weeks before intestinal perforation. At that time, the

clinicians did not suspected WG, and treatment was

delayed. It has been suggested that severe intestinal

involvement improved after treatment with immunosup-

pressive drugs in patients with WG [7]. In this case, it can

be speculated that early treatment with immunosuppressive

drugs may be prevented severe intestinal perforation.

In conclusion, WG should be suspected in patients with

chronic, unexplained respiratory symptoms and signs. Also

the clinicians should be considered WG in the differential

diagnosis of intestinal perforation.

References

1. Storesund B, Gran JT, Koldingsnes W (1998) Severe intestinal

involvement in Wegener’s granulomatosis: report of two cases and

review of the literature. Br J Rheumatol 37:387–390

2. Walton EW (1958) Giant-cell granuloma of the respiratory tract

(Wegener’s granulomatosis). Br Med J 2:265–270

3. Akca T, Colak T, Caðlykulekci M, Ocal K, Aydyn S (2005)

Intestinal perforation in Wegener’s granulomatosis: a case report.

Ulus Travma Derg 11:348–351

4. Storesund B, Gran JT, Koldingsnes W (1998) Severe intestinal

involvement in Wegener’s granulomatosis: report of two cases and

review of two cases and review of the literature. Br J Rheumatol

37:387–390

5. Demling RH, Salvatierra O, Belzer FO (1975) Intestinal necrosis

and perforation after renal transplantation. Arch Surg 110:251–253

6. Yildirim AC, Kocak E, Yildiz P, Yildiz M, Karakayali AS,

Kaptanoglu B, Koklu S (2010) Multiple intestinal perforation in a

patient with Wegener’s granulomatosis: a case report and review

of the literature. Gastroenterol Clin Biol 34:712–715

7. Haworth SJ, Pusey CD (1984) Severe intestinal involvement in

Wegener’s granulomatosis. Gut 25:1296–1300

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