Imaging Findings of Cerebral Amyloid Angiopathy, Abeta-Related Angiitis (ABRA) and Cerebral Amyloid Angiopathy-Related Inflammation: A Single Institution 25 Year Experience

Carlo Salvarani¹, Jonathan M Morris², Caterina Giannini², Teresa J. H. Christianson³, Robert D. Brown Jr.⁴ and Gene G. Hunder⁵, ¹Rheumatology, Arcispedale S Maria Nuova-IRCCS, Reggio Emilia, Italy, ²Mayo Clinic, Rochester, MN, ³Division of Biomedical Statistics & Informatics, Mayo Clinic, Rochester, MN, ⁴Department of Neurology, Mayo Clinic, Rochester, MN, ⁵Dept Internal Med/Rheum, Mayo Clinic, Rochester, MN

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: CNS Vasculitis

Session Information

Date: Monday, November 9, 2015

Title: Vasculitis Poster II

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose: To investigate the role of imaging in differentiating cerebral amyloid angiopathy (CAA) with and without inflammation.

Methods: We studied 54 patients seen over 25 years with pathological evidence of CAA with or without vascular inflammation and with available neuroimaging at the time of diagnosis. All pathological specimens were reviewed by one neuropathologist to evaluate the presence and distribution of inflammation associated with amyloid. All available neuroimaging findings were reviewed by a neuroradiologist. Clinical data were recorded

Results: Radiologic findings at diagnosis were available in 27 patients with CAA without inflammation, 22 with Aβ-related angiitis (ABRA) and 5 with CAA-related inflammation (CAA-RI). On MRI, leptomeningeal disease alone or with infiltrative white matter was significantly more frequent at presentation in patients with ABRA or CAA-RI compared to those with CAA (8 of 27, 29.6% vs 1 of 27, 3.7%, p = 0.02; and 11 of 27, 40.7% vs 1 of 27, 3.7%, p = 0.002, respectively), while lobar hemorrhage was more frequent in patients with CAA (17 of 27, 62.3% vs 2 of 27, 7.4%, p = 0.0001). Overall, leptomeningeal involvement at presentation was present in 70.4% (19 of 27) of patients with ABRA or CAA-RI and in only 7.4% (2 of 27) of patients with CAA (p = 0.0001). The sensitivity and specificity of leptomeningeal enhancement to identify patients with ABRA or CAA-RI were 70% and 93% respectively, while the positive likelihood ratio (LR) was 9.5. The sensitivity and specificity of intracerebral hemorrhage to identify patients with CAA were 63% and 93% respectively, while the positive LR was 8.5. Prior microhemorrhages at the grey white junction on GRE and/or SWI sequences were more frequently observed in patients with ABRA or CAA-RI compared to those with CAA (19 of 27, 70.4% vs 10 of 27, 37%, p = 0.03).

Conclusion: Leptomeningeal enhancement and lobar hemorrhage at presentation may enable differentiation between CAA with and without inflammation. SWI or GRE image aid in determining the correct diagnosis of ABRA or CAA-RI and aid in the decision regarding treatment.

Disclosure: C. Salvarani, None; J. M. Morris, None; C. Giannini, None; T. J. H. Christianson, None; R. D. Brown Jr., None; G. G. Hunder, None.

To cite this abstract in AMA style:

Salvarani C, Morris JM, Giannini C, Christianson TJH, Brown RD Jr., Hunder GG. Imaging Findings of Cerebral Amyloid Angiopathy, Abeta-Related Angiitis (ABRA) and Cerebral Amyloid Angiopathy-Related Inflammation: A Single Institution 25 Year Experience [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/imaging-findings-of-cerebral-amyloid-angiopathy-abeta-related-angiitis-abra-and-cerebral-amyloid-angiopathy-related-inflammation-a-single-institution-25-year-experience/. Accessed .

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