Wegener granulomatosis with spleen infarction: case report and review of the literature

Abstract

Objective: To report a case of biopsy-verified Wegener granulomatosis (WG) with positive c-antineutrophil cytoplasmic antibodies who had evidence of spleen infarction and to review the relevant literature on spleen involvement in WG.

Methods: Descriptive case report of 1 patient with WG complicated by spleen infarction and review of the relevant literature (PubMed search 1966 through February 2007).

Results: In our patient spleen involvement (infarction) was clinically silent and was incidentally detected on the caudal slides of a chest computed tomography (CT) performed to investigate pulmonary involvement. Similar cases have been reported: in the published literature reviewed, overall 18 patients have been described and other cases have been described after autopsy. Of these, 8 patients were symptomatic: generally, they complained of diffuse or left upper quadrant abdominal pain. In 15 patients, spleen involvement was detected by ultrasound, scintigraphy, CT scan, or magnetic resonance imaging. In 1 case only was the diagnosis confirmed by biopsy (after splenectomy).

Conclusions: The frequency of spleen infarction is probably underestimated in WG since it is often clinically silent as in our patient. In addition to spleen infarction, WG has also been implicated in inducing spleen hemorrhage, capsular adhesions, dysfunction, and splenomegaly. Since some of the spleen lesions may lead to complications, a high index of suspicion should be maintained for spleen involvement in patients with WG, particularly in the presence of abdominal symptoms.