Cerebral vasculitis
Cerebral vasculitis | |
---|---|
Other names | Central nervous system vasculitis |
Specialty | Cardiology, neurology, rheumatology |
Cerebral vasculitis (sometimes the word angiitis is used instead of "vasculitis") is
Causes
"Primary" angiitis/vasculitis of the central nervous system (PACNS) is said to be present if there is no underlying cause. The exact mechanism of the primary disease is unknown, but the fundamental mechanism of all vasculitides is
Diagnosis
Treatment
Treatment is first with many different high-dose steroids, namely glucocorticoids. Then, if symptoms do not improve additional immunosuppression such as cyclophosphamide are added to decrease the immune system's attack on the body's own tissues.[3] Cerebral vasculitis is a very rare condition that is difficult to diagnose, and as a result there are significant variations in the way it is diagnosed and treated.[5]
Specific Diseases
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Giant cell arteritis (GCA)
(Also known as temporal arteritis)
Symptoms
- Fever
- general uneasiness
- weight loss
- inflammation of the muscles causing stiffness in the shoulders; neck; and/or upper arms
- persisting headache
- pain in the jaw or ear while eating
- double vision
- partial loss of vision or blind spots
- (on rare occasions) stroke.
Diagnostic criteria
Three or more of the following five criteria must be met:
- Age 50 years or more
- New developed headache
- Tenderness of the superficial temporal artery
- Elevated sedimentation rate, at least 50 mm/hour (blood test that reveals inflammatory activity)
- Giant cell arteritis in a biopsy specimen from the temporal artery
Takayasu's arteritis
Symptoms
Starts with nonspecific symptoms such as:
- Localized joint pain
- Fever
- Fatigue
- Headaches
- Rashes
- Weight loss
- Diagnosis usually does not happen until the blockage causes deficient blood flow to the extremities or to a stroke.
Classification criteria
Three or more of the following six criteria must be met:
- Age when disease starts is under 50
- Decreased brachial artery pulse
- Systolic blood pressure differs by more than 10mmHg between arms
- Cramping caused by exercise in the extremities
- Abnormal sounds (through stethoscope) over subclavian arteries or abdominal aorta
- A narrowing or blockage in the aorta, its primary branches, or large arteries as seen through a radiograph of the arteries.
Treatment therapy
- 50% of patients respond to corticosteroid therapy alone in early phases
- Methotrexate or Azathioprine are an alternative to corticosteroid immunosuppressants
- There have been studies on Mycophenolate mofetil and anti-TNF therapies
- In Takayasu's arteritis it is vital to combine drug treatments often with low-dose aspirin or statin
Polyarteritis nodosa (PAN)
Symptoms
- Systemic illness with fever
- General feeling of discomfort or uneasiness with cause difficult to identify
- Weight loss
- Arthritis
- Black discoloration of skin primarily on the extremities
- Severe inadequate blood supply to the extremities
- Ischemic stroke, hemorrhages and a progressive encephalopathy with or without seizures may occur
Diagnostic Criteria
Three or more of the following ten criteria are required:
- More than 4 kg (8.8 lb) weight loss
- Lace-like purplish discoloration of the skin (livedo reticularis)
- Testicular pain
- Pain in a muscle or group of muscles (myalgias)
- Damage to peripheral nerves
- Elevation of blood pressure by more than 90 mmHg
- Creatinine serum levels greater than 1,5 mg/dl
- Hepatitis B or C virus antibodies
- An aneurysm or occlusion as shown in a pathologic arteriography
- Histology findings typical of PAN
Treatment therapy
- In PAN not associated with a hepatitis virus: prednisone and cyclophosphamide therapy. In case of emergency, plasmapheresis may be tried
- In PAN associated with a hepatitis virus: combination therapy of
Granulomatosis with polyangiitis (GPA)
Symptoms
- Men are affected twice as often as women
- Compression of structures surrounding the nose and paranasal sinuses
- Diabetes insipidus
- Abnormal protrusion of the eyeball(s)
- Nonseptic meningitis
- Affection of the lung and kidney due to destruction of the arteries and veins
- Ischemic stroke, hemorrhages, or encephalopathy with possible seizures
Diagnostic Criteria
Two or more of the following four criteria are required:
- Necrotizing ulcerating inflammation of nose, sinuses, mouth or pharynx
- Irregular lung infiltrates
- Nephritis
- Granulomatous vascular and perivascular inflammation
Treatment Therapy
- Corticosteroids (e.g., Prednisolone)
- Cyclophosphamide
- Azathioprine
- Mycophenolate mofetil
References
- ^ "Cerebral Vasculitis". Prime Health Channel. 19 December 2012. Retrieved 1 May 2015.
- PMID 9093590.
- ^ PMID 11198690. Archived from the original(PDF) on 2009-11-05. Retrieved 2008-07-23.
- ^ "Central Nervous System Vasculitis (CNS Vasculitis)". Cleveland Clinic. Retrieved 1 May 2015.
- S2CID 18859529.
- PMID 21180634.