Aneurysms may be more frequent in patients with granulomatosis with polyangiitis (GPA) compared to other types of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and predominantly affect medium-sized vessels, in particular the renal arteries, according to a retrospective multicenter study and systematic literature review published in Autoimmunity Reviews.
“The mortality associated with aneurysmal disease is significant, particularly in the case of rupture, and specific treatment should be proposed,” the study’s authors said. “It would seem interesting to better define the pathophysiological substrate underlying aneurysmal formation in ANCA vasculitides to better characterize these disorders and ultimately propose targeted treatments.”
The analysis of 51 patients with AAV revealed a high risk of aneurysmal rupture. Twenty-two patients suffered from aneurysmal rupture during follow-up (median follow-up, 24 months), with most (91%) showing medium-sized vessel involvement. Patients with aneurysmal rupture had more lung infiltrates and AAV-related ocular involvement, but less skin and peripheral nerve involvement than patients without rupture.
Seventeen patients experienced relapses during follow-up (71% had GPA) and 10 patients died. Causes of death included infection (n=5), aneurysmal rupture (n=3), and vasculitis-related heart failure (n=1).
Almost all (n=49) patients enrolled in the study received glucocorticoids and 39 patients received cyclophosphamide. Additional treatments included rituximab (n=2), azathioprine or methotrexate for induction (n=2), and plasma exchange (n=4). Twenty-two patients initiated maintenance treatment.
Moreover, 10 patients with aneurysmal rupture were treated with either endovascular treatments (n=7) or surgical vascular repair (n=3), whereas 8 patients did not receive surgical or endovascular treatment.
The study cohort included 5 patients from different French centers, 10 patients from the French Vasculitis Study Group database, and 36 patients retrieved from the literature. All patients were diagnosed with AAV, mostly in the form of GPA (67%). The proportion of patients with microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis was 29% and 4%, respectively. In total, the study encompassed 92 aneurysms.
Hankard A, Puéchal X, Martin Silva N, et al. Characteristics of ANCA-associated vasculitis with aneurysms: case series and review of the literature. Autoimmun Rev. 2023;22(5):103293. doi:10.1016/j.autrev.2023.103293