Aβ-related angiitis: comparison with CAA without inflammation and primary CNS vasculitis.
OBJECTIVE
To analyze the clinical findings, response to therapy, and outcomes of patients with cerebral vascular amyloid-β (Aβ) deposition with and without inflammatory vascular infiltration.


METHODS
We report 78 consecutive patients with cerebral vascular Aβ deposition examined at Mayo Clinic Rochester over 25 years (1987 through 2011). Specimens reviewed by a neuropathologist showed 40 with vascular Aβ peptide without inflammation (cerebral amyloid angiopathy [CAA]), 28 with granulomatous vasculitis (Aβ-related angiitis or ABRA), and 10 with perivascular CAA-related inflammation. We also matched findings in 118 consecutive patients with primary CNS vasculitis (PCNSV) without Aβ seen over 25 years (1983 through 2007).


RESULTS
Compared to the 40 with CAA, the 28 with ABRA were younger at diagnosis (p = 0.05), had less altered cognition (p = 0.02), fewer neurologic deficits (p = 0.02), and fewer intracranial hemorrhages (<0.001), but increased gadolinium leptomeningeal enhancement (p = 0.01) at presentation, and less mortality and disability at last follow-up (p < 0.001). Compared with PCNSV, the 28 patients with ABRA were older at diagnosis (p < 0.001), had a higher frequency of altered cognition (p = 0.05), seizures/spells (p = 0.006), gadolinium leptomeningeal enhancement (p < 0.001), and intracerebral hemorrhage (p = 0.02), lower frequency of hemiparesis (p = 0.01), visual symptoms (p = 0.04), and MRI evidence of cerebral infarction (p = 0.003), but higher CSF protein levels (p = 0.03). Results of treatment and outcomes in ABRA and PCNSV were similar.


CONCLUSIONS
ABRA appears to represent a distinct subset of PCNSV.
From the Unità Operativa di Reumatologia (C.S.), Azienda Ospedaliera ASMN, Istituto di Ricovero e Cura a Carattere Scientifico, Reggio Emilia, Italy; and Anatomic Pathology, Department of Laboratory Medicine and Pathology (C.G.), Departments of Radiology (J.M.M.) and Neurology (R.D.B.), and Divisions of Biostatistics (T.C.) and Rheumatology (G.G.H.), Mayo Clinic, Rochester, MN.
Carlo
C
Salvarani
Gene G
GG
Hunder
Jonathan M
JM
Morris
Robert D
RD
Brown
Teresa
T
Christianson
Caterina
C
Giannini
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D016229:Amyloid beta-Peptides / Q000378:metabolism*; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D016657:Cerebral Amyloid Angiopathy / Q000150:complications / Q000175:diagnosis* / Q000378:metabolism* / Q000628:therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D017063:Outcome Assessment, Health Care; D016016:Proportional Hazards Models; D016896:Treatment Outcome; D020293:Vasculitis, Central Nervous System / Q000150:complications / Q000175:diagnosis* / Q000378:metabolism* / Q000628:therapy; D055815:Young Adult
D016229:Amyloid beta-Peptides; D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide