Vasculitis

 

- angiography is suggestive but not diagnostic of CNS vasculitis

- CNS vasculitis is very rare

 

Vasculitis - inflammation and necrosis of blood vessels. Can thrombose or rupture

 

Classified by vessel size (large, medium, small).

 

Large vessel

- giant cell arteritis

- Takayasu's arteritis

Most common is Giant cell arteritis. Keep in mind geographic variation, none in Asia.

 

Giant cell arteritis, diagnostic features:

- New onset headache

- Jaw claudication

- Diplopia

- 90% have ESR >40

Biopsy diagnostic even months after treatment. Treat with steroids (prednisone 1mg/kg/day for months, then taper)

 

Takayasu's arteritis

- women < 40y.o., not necessarily Asian, most common is left subclavian

- easy to diagnose radiologically, complicated to treat: symptoms nonspecific, ischemic findings vague, inflammatory markers may not be concordant

- usually long delay in diagnosis, often absent pulse, inability to obtain BP, limb claudication, TIAs (esp ocular)

 

Medium vessel

- Kawasaki

- isolated CNS vasculitis

- PAN (polyarteritis nodosa)

Mononeuritis multiplex, if not diabtetic, mostly caused by medium and small vessel vasculitis

 

Microscopic polyangitis - PAN plus pulmonary and/or renal involvement

 

Small vessel

- ANCA-related

            - Wegener

            - Churg-Strauss vasculitis

            - drugs - PTU

            - microscopic polyangiitis

- non-ANCA

            - IBD

            - lymphoproliferative disorders

            - immune complex, e.g., lupus, viruses, HSP, drug

 

Not classified (vasculitis?)

- Behçet's

- Moyamoya

- Burger's

 

CNS vasculitis

- in presence or absence of systemic vasculitis

 

Secondary

- PAN

- Behçet's

- Wegener's

- Churg-Strauss

- Takayasu's

- Connective tissue diseases (SLE, Sögren's Syndrome)

 

Primary CNS vasculitis

- PACNS - primary angiitis of CNS

- BACNS - benign angiopathy of CNS

 

PACNS

            - male predominance 70%

            - mean age 46 +/- 17

            - long prodrome

            - assoc decreased cognition

            - can have mass lesion, spinal cord involvement, CNS hemorrhage

 

BACNS

            - female predominance 70%

            - mean age 33 +/- 14

            - acute

 

DIAGNOSIS OF EXCLUSION

R/O drugs, infections, neoplasms, vasospastic disorders, vasculopathies, demyelinating diseases, neurofibromatosis

--> must do CSF studies

 

MRI has strong negative predictive value. But MRI does not correlate with angiography - do both if suspect. Brain biopsy useful to R/O mimickers.

 

Treatment

PACNS - corticosteroids + methotrexate x1mo then 6mo taper

BACNS - corticosteroids (6mo) + verapamil (lifelong)

 

Relevant Reading

Calabrese, Vascuilitis in CNS, Arthritis Rheum, 1997