Brief report
Arterial Aneurysms in Wegener’s Granulomatosis: Case Report and Literature Review

https://doi.org/10.1016/j.semarthrit.2007.07.004Get rights and content

Objective

Arterial aneurysms are characteristic of medium-size vessel vasculitis but are a very unusual feature of Wegener’s granulomatosis (WG). We describe a typical WG case, complicated by arterial aneurysms and review previously reported cases.

Methods

Medline database search of cases published between January 1978 and July 2006, in English, reporting arterial aneurysms complicating WG.

Results

Five years after diagnosis, a 29-year-old man with typical WG developed macro- and microaneurysms located on branches of the hepatic and renal arteries during a disease relapse. The main symptoms were abdominal pain, vomiting, and altered general status. He was successfully treated by coil embolization in combination with prednisone, intravenous mycophenolate mofetil, and high-dose immunoglobulins. Twelve additional cases of WG complicated by arterial aneurysms are reported in the English literature. This represents a life-threatening complication since rupture occurred in half of the patients.

Conclusions

Although small-vessel injury predominates in WG, inflammation of medium-size arteries may occur and lead to aneurysm formation. Abdominal angiography should be recommended when unexplained abdominal pain occurs during a WG flare.

Section snippets

Methods

A literature survey from January 1978 to July 2006 was performed using a MEDLINE (PubMed), searching for cases with coexisting WG and aneurysm. Only cases published in English with sufficient clinical data for analysis were reviewed.

Case Report

An Algerian man, born in 1973, was admitted to our department of internal medicine in January 2003, because of weight loss, vomiting, and epigastric pain. He had been diagnosed with WG in May 1997 on the basis of nasal ulcerations with epistaxis, nasal perforation and saddle-nose deformity, sinusitis, orchitis, polyarthralgias, myalgias, subcutaneous nodules, polyneuropathy, and acute renal insufficiency related to pauci-immune crescentic glomerulonephritis. Histological examination of skeletal

Discussion

We report an unusual and severe case of WG with multiple aneurysms located on medium-sized arteries. The diagnosis of WG was based on typical characteristics: rhinitis and sinusitis, pauci-immune crescentic glomerulonephritis, intraalveolar hemorrhage, and polyneuropathy. Histological studies showed giant-cell granuloma and/or necrotizing granulomatous inflammation of medium and small vessels in the skin, muscle, and gastrointestinal tract. Anti-PR3 antibodies were present.

Aneurysms are an

Acknowledgment

We thank Jacques Pouchot, MD, for critically reviewing the manuscript.

References (14)

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The authors have no conflicts of interest to disclose.

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