Systemic multiple aneurysms are rare findings in granulomatosis with polyangiitis (GPA).1
A 66-year-old woman presented with generalized, subcutaneous nodules and a right leg ulcer of 1-month duration. She had a history of multiple pulmonary nodules, initially diagnosed as lymphomatoid granulomatosis, for which she had received prednisolone therapy until 8 years ago. Her serum creatinine and urinalysis findings were normal. Proteinase 3–antineutrophilic cytoplasmic antibody level was 5.7 U/mL (reference range: 0-3.5 U/mL). Computed tomography (CT) of the chest demonstrated pulmonary nodules and 3-D CT angiography (CTA) showed aneurysms in the branches of the celiac and superior mesenteric arteries. 3-D CTA of the head demonstrated anterior and middle cerebral artery aneurysms (Figure 1A). A skin biopsy of the lesions and a review of a previously performed lung biopsy found fibrinoid necrosis with vasculitis and granuloma (Figure 2). Based on these findings, GPA was diagnosed. Treatment with systemic glucocorticoids and rituximab was begun. At a 1-year follow-up visit, the patient’s symptoms had improved, and the brain artery aneurysms had almost disappeared in 3-D CTA (Figure 1B). Although aneurysms of medium-sized vessels have been reported in GPA, these rarely occur in the cerebral arteries, and treatment is essential to prevent their rupture.2
3-D CTA of cerebral arteries. (A) 3-D CTA shows multiple aneurysms (arrows) in cerebral arteries before treatment. (B) The aneurysms disappeared after 1-year treatment. CTA: computed tomographic angiography; P: posterior; R: right.
Left lung lower lobe biopsy demonstrating necrotizing vasculitis with fibrinoid necrosis and adjacent granuloma formation.
Footnotes
The authors declare no conflicts of interest relevant to this article. The Tokyo Metropolitan Tama Medical Center ethics committee approved the protocol and informed consent was obtained using an opt-out approach.
- Copyright © 2024 by the Journal of Rheumatology
