Background/Purpose:

Amyloid β-related angiitis (ABRA) is a rare type of Central Nervous System Vasculitis (CNSV), which complicates Cerebral Amyloid Angiopathy (CAA).  Data about clinical features, management and outcomes of ABRA are scant. It is important to differentiate this treatable disease from primary CNSV and other irreversible neurological disorders.

Methods:

We searched the OVID Medline database for relevant articles between 1946 to 2012 using the  key words “Cerebral amyloid angiopathy”, “vasculitis” “inflammatory CAA” “Amyloid Beta-Related Angiitis”.    Data from major case series and reviews as well as individual case reports was extracted.  We found total 98 patients who were reported in the literature. Two case reports were in other languages and data was unavailable for 2 other patients.  Demographics, clinical features, lab and imaging findings, treatment and follow up was extracted for 94 patients  and is  summarized descriptively.

Results:

Patients with mean age of 67 years presented acutely (54%) or subacutely (46%) with cognitive or behavioral changes of variable severity (71%), focal deficits (51%), seizures (36%) and headache (35%). More than 2 features were present in 77%. Prior malignancy was reported in 10 patients and associated autoimmune disease (e.g. autoimmune thyroiditis, autoimmune hepatitis, RA and pernicious anemia) in 11. ESR and CRP were elevated in 14 /47 (29.7%); 70% patients (12/17) had ApoE genotype e4/e4   CSF showed elevated protein in 71% (42/59) and lymphocytic pleocytosis in 44% (26/ 59) and abnormalities normalized after treatment in 2/2 patients.  Brain MRI was abnormal in 97.4 % (75/77) and typically showed asymmetrical bilateral white matter hyperintensity on T2W and FLAIR (88%), cortical microbleeds in 87% (21/24) on SWI and minimally enhancing mass like lesions (26%).  Post treatment MRI showed improvement in 38/47 (80%) patients. Brain biopsy confirmed diagnosis in 97.8% (92/94) patients which showed transmural inflammation with deposition of A Β-amyloid in vessels in 80% and pervasculitis in 20% patients. Of 73 patients who received corticosteroids 78% showed either partial or complete clinical improvement. Cyclophosphamide (34%), azathioprine (6.6%), methotrexate (2%) and mycophenolate (2%) were also used successfully in conjunction with steroids. Relapse occurred in 26% of 57 patients, either after reduction or cessation of therapy and responded well after retreatment. 

Conclusion:

ABRA is a rare, but treatable cause of progressive dementia and neurological dysfunction in elderly and should be considered in the differential diagnosis of rapid onset neurological disease . It has characteristic MRI findings and responds well to steroids and immunosuppressants. 

Study	Scolding et al	Kinnecom et al	Salvarani et al	Chung et al	Others	Total
No of patients	34	14	8	16	22	94
Age	67.3 (43-82)*	63.2 (45-79)*	63 (42-84) *	69 (46-83)**	67.6 (52-87)*	65.2 (42-87)*
Sex (M/F)	17/17	9/5	6/2	7/9	11/11	50/44
Clinical features CB change Seizures Focal deficits Headache	29/34 8/34 15/34 12/34	9/12 7/12 1/12 6/12	6/8 0/8 7/8 4/8	9/16 7/16 9/16 7/16	13/22 12/22 15/22 4/22	66/92 (71%) 34/92 (36%) 47/92 (51%) 33/92 (35%)
ApoE e4/e4	NA	10/13	NA	NA	2/4	12/17 (70%)
CSF Elevated proteins Pleocytosis Oligoclonal bands Repeat CSF	11/18 8/18 12/21	4/5 1/5 NA	7/8 5/8 NA	8/11 5/11 1/1	12/17 7/17 3/3	42/59 (71%) 26/59 (44%) 16/16(100%)
MRI White matter changes# Microbleed on SWI/GEI Enhancement Improvement with Rx	13/20 4 7/10 4/5	12/12 6/7 NA 10/12	6/8 NA 5/8 4/6	14/14 4/4 5/9 6/9	21/21 11/13 6/11 13/15	66/75(88%) 21/24(87%) 23/38 (60%) 37/47 (79%)
Pathology Vasculitis or perivasculitis	34/34	14/14	8/8	16/16	19/20‡‡	91/92 (99%)
Response to therapy	12/20	10/12	6/8	10/12	19/20	57/72 (79%)
Relapse	NA	3/12	2/8	NA	3/14∞	8/34 (23%)

*Mean (range) age     **Median (range) age

# Hyperintesnsities on T2W or FLAIR 

SWI Susceptibility weighted imaging

CB- Cognitive behavioral  

‡‡ 2 patients were treated presumptively

∞ As per the information wherever it was reported

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