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

• Published in: Seminars in arthritis and rheumatism 2008-02, Vol.37 (4), p.265-268
• Main Authors: Arlet, Jean-Benoit, MD, Le Thi Huong, Du, MD, PhD, Marinho, Antonio, MD, Cluzel, Philippe, MD, Wechsler, Bertrand, MD, Piette, Jean-Charles, MD
• Format: Article
• Language: English
• Subjects: Adult; aneurysm; Aortic Aneurysm, Abdominal - etiology; Biological and medical sciences; Gastroenterology. Liver. Pancreas. Abdomen; Granulomatosis with Polyangiitis - complications; Hepatic Artery; Humans; intestinal perforation; intravenous immunoglobulins; Male; Medical sciences; Other diseases. Semiology; Renal Artery; Rheumatology; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Stomach. Duodenum. Small intestine. Colon. Rectum. Anus; Wegener’s granulomatosis
• ISSN: 0049-0172; 1532-866X
• DOI: 10.1016/j.semarthrit.2007.07.004

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.