CNS (Central Nervous System) Vasculitis

CNS (central nervous system) vasculitis is a type of vasculitis. It causes inflammation in the blood vessels that supply blood to your brain and spinal cord. Healthcare providers sometimes call it primary angiitis of the central nervous system or granulomatous angiitis.

Your central nervous system is the part of your nervous system that regulates everything your body does. It oversees movements and actions you’re aware of (like using your fingers to scroll through this article) and ones you don’t control (like breathing and your heart beating).

Types of CNS vasculitis

Healthcare providers classify CNS vasculitis as either primary or secondary:

• Primary CNS vasculitis. Primary CNS vasculitis happens on its own without other conditions causing or triggering it.
• Secondary CNS vasculitis. Secondary is the medical term for something happening alongside or because of another issue. Conditions that cause inflammation can trigger CNS vasculitis. Autoimmune diseases and infections are the most common secondary CNS vasculitis triggers.

Symptoms of CNS vasculitis

Symptoms of CNS vasculitis can include:

• Severe headaches that last a long time
• Forgetfulness or confusion
• Tingling, numbness or changes in your sense of touch (sensations)
• A feeling of weakness
• Worsening vision (including blurred vision or double vision)
• Seizures
• Encephalopathy (brain swelling) that can cause mood or personality changes or make you faint (pass out)

CNS vasculitis complications

If you have vasculitis, your blood vessels swell and thicken. This makes it harder for blood to flow through them. Over time, the inflammation from CNS vasculitis can damage your brain or spinal cord and cause serious, potentially life-threatening complications, including:

• Strokes
• Transient ischemic attacks (“mini-strokes”)
• Brain aneurysms

These issues are all medical emergencies. Go to the emergency room or call 911 (or your local emergency services number) if you think you’re experiencing symptoms that might be a stroke or a brain aneurysm rupture.

CNS vasculitis causes

CNS vasculitis is an autoimmune disease. Autoimmune diseases happen when your immune system accidentally attacks your body instead of protecting it.

People with primary CNS vasculitis develop it without a known cause or trigger (idiopathically).

Secondary CNS vasculitis usually develops alongside (secondary to) conditions that cause inflammation in your body. Other autoimmune diseases and certain infections can trigger it, including:

• Lupus
• Granulomatosis with polyangiitis
• Eosinophilic granulomatosis with polyangiitis (EGPA)
• Microscopic polyangiitis
• Behçet’s syndrome
• Sjögren’s syndrome
• Viral infections (like Epstein-Barr virus and varicella-zoster virus)
• Bacterial infections (like tuberculosis and syphilis)

Risk factors of central nervous system vasculitis

Anyone can develop CNS vasculitis, but it’s more common in males. It’s most likely to develop in people around age 50, but can occur at any age.

How do healthcare providers diagnose CNS vasculitis?

A healthcare provider will diagnose CNS vasculitis with a physical exam and some tests. They’ll ask you about your symptoms and when you first noticed them. Tell your provider if the symptoms come and go (recur) or if certain times of day or activities make them better or worse.

Diagnosing CNS vasculitis can be a differential diagnosis. This means your provider will probably use a few tests to rule out other, more common conditions before diagnosing you with CNS vasculitis. You may need:

• MRI
• CT scan
• Angiogram
• Biopsies

You might need to see a rheumatologist — a healthcare provider who specializes in treating inflammatory diseases.

CNS vasculitis markers

Markers are signs of a disease that your provider can look for in your blood or body using laboratory tests. Tests your provider may use to check you for markers of CNS vasculitis include:

• Blood tests to look for signs of anemia
• An ESR test to look for markers that indicate inflammation in your body
• A C-reactive protein (CRP) test to look for inflammation markers that your liver releases
• A lumbar puncture (spinal tap) to examine your cerebral spinal fluid for a high white blood cell count

CNS vasculitis treatments

Your provider will suggest treatments that manage inflammation. The most important part of treating CNS vasculitis is reducing how much it damages your brain or spinal cord. Most people need a combination of corticosteroids and immunosuppressants.

You might need higher doses of medication at first. Then (usually after a few months), your provider may reduce how often you receive infusions or injections of medication. They’ll adjust your medications to help you reach a remission stage — a long period where you have very mild (or no) symptoms.

If you have secondary CNS vasculitis, you may need other treatments to manage the condition that triggered it. Your provider will tell you what to expect.

When should I see my healthcare provider?

Monitor the symptoms you’re experiencing and keep track of any changes you notice. Talk to your provider if you notice any changes, especially if it feels like symptoms are happening more often or more severely. Tell your provider if it feels like a treatment isn’t working (or isn’t as effective as it used to be).

Your provider will tell you how often you’ll need regular follow-up appointments, blood tests or additional screenings.

Go to the emergency room or call 911 (or your local emergency number) if you’re experiencing any of the following symptoms:

• You think you might be experiencing stroke or brain aneurysm rupture symptoms.
• You can’t breathe.
• Your vision suddenly gets worse or you have sudden vision loss.

What can I expect if I have central nervous system vasculitis?

There’s no cure for CNS vasculitis. Many people are able to enter extended periods of remission once they find the right medications that work for them. Some people are in remission for months or years before experiencing symptoms again.

CNS vasculitis doesn’t usually affect how long you can expect to live (your life expectancy). But this can be hard to estimate. That’s because CNS vasculitis can cause severe complications like strokes and aneurysms. People who experience those complications are much more likely to die sooner than usual.

Talk to your healthcare provider if you’re worried about your health or how much having CNS vasculitis can affect it.