Vasculitis And Stroke: Understanding The Potential Risk

Vasculitis is a rare condition characterised by inflammation of the blood vessel walls. It can affect vessels of any size and can be caused by a variety of conditions and illnesses. The inflammation can cause the blood vessel walls to weaken, stretch, thicken, swell or develop scarring, which can slow or completely stop the normal flow of blood. This can lead to a loss of brain function and, in the case of cerebral vasculitis, a stroke.

Vasculitis and its role in causing strokes in young people

Vasculitis is an inflammation of the blood vessels that carry blood through the body. It occurs when the body's immune system mistakenly attacks its own blood vessels. This can be due to an infection, medication, another disease, or unknown causes. Vasculitis can affect blood vessels of any type, size, or location, including those in the brain and spinal cord. While vasculitis can occur at any age, certain forms of the disease are more common in younger individuals.

The role of vasculitis in causing strokes in young people

Vasculitis is a rare but important cause of stroke in young people, accounting for _3% of strokes in patients under 50 years of age. It is one of the main causes of stroke in young adults, along with other factors such as hypertension, hypercholesterolemia, obesity, and diabetes mellitus. The inflammation caused by vasculitis can lead to the weakening and rupture of blood vessels in the brain, resulting in bleeding into surrounding tissues (hemorrhagic stroke) or the formation of blood clots (ischemic stroke). Ischemic strokes are the most common type of stroke in young people.

Young people with vasculitis may experience a range of symptoms, including headaches, encephalopathy, seizures, stroke-like episodes, and cognitive impairments. The diagnosis of vasculitis-related stroke can be challenging and often requires a comprehensive approach involving neurological examination, imaging techniques such as MRI and CT scans, lumbar puncture, and, in some cases, brain biopsy.

The treatment for vasculitis-related stroke involves immunosuppressive therapy, including steroids and other immunosuppressants. Early diagnosis and prompt treatment are crucial to improve outcomes and reduce the risk of long-term disabilities associated with stroke in young people.

How to identify and manage vasculitis

Vasculitis is an autoimmune disease that causes inflammation and swelling in blood vessels, which can lead to restricted blood flow and organ damage. While vasculitis is a rare condition, it can be life-threatening and requires careful management. Here are some detailed instructions on how to identify and manage this condition:

Identifying Vasculitis:

• Vasculitis can affect blood vessels of any type, size, or location, including arteries and veins. It causes inflammation and swelling, leading to restricted blood flow.
• Common symptoms of vasculitis include numbness or tingling, especially in the hands and feet. Rashes, bumps, or skin discolouration may also be present.
• Other symptoms include fatigue, abdominal pain, kidney problems (such as dark or bloody urine), fever, weight loss, joint pain, and headaches.
• To diagnose vasculitis, a doctor will review the patient's medical history, perform a physical examination, and order diagnostic tests such as blood and urine tests, imaging scans, and, in some cases, a biopsy of the affected tissue.

Managing Vasculitis:

• Most people with vasculitis can manage their symptoms with medication. Immunosuppressants or biologics are often prescribed to control inflammation and manage symptoms.
• In some cases, surgery may be necessary to treat damaged blood vessels or aneurysms.
• Vasculitis cannot be cured, but with proper treatment, it can go into remission, which means long periods of time between episodes of symptoms.
• It is important to monitor symptoms closely and keep track of any changes. Regular follow-up appointments with a healthcare provider are essential to manage vasculitis effectively.
• Lifestyle changes, such as quitting smoking and avoiding illegal drugs, are also recommended to lower the risk of heart disease and other complications.

Types of vasculitis: primary vs secondary

Vasculitis is a group of disorders that destroy blood vessels by inflammation. Both arteries and veins are affected. There are many different types of diseases that belong to this category, and they are often different in terms of which organs are affected, medications used to treat them, and other characteristics.

Primary Vasculitis

Primary vasculitis, also known as primary systemic vasculitis, is further categorized by the size of the vessels mainly involved. The primary systemic vasculitis includes:

• Large-vessel vasculitis (LVV): LVV affects the largest-sized blood vessels of the body, such as the aorta and major arteries that deliver blood to distant parts of the body. Examples include Giant cell arteritis and Takayasu arteritis.
• Medium-vessel vasculitis (MVV): MVV involves the inflammation of medium-sized blood vessels, including arterioles and smaller veins. Examples include Polyarteritis nodosa and Kawasaki disease.
• Small-vessel vasculitis (SVV): SVV affects the smallest blood vessels, called capillaries and venules. Examples include Microscopic polyangiitis, Granulomatosis with polyangiitis, and Eosinophilic granulomatosis with polyangiitis.
• Variable-vessel vasculitis (VVV): VVV can affect blood vessels of any size, type, or location. Examples include Behcet's disease and Cogan's syndrome.
• Single-organ vasculitis (SOV): SOV involves inflammation of the blood vessels supplying a single organ, such as the brain or skin.

Secondary Vasculitis

Secondary vasculitis is a subset of illnesses believed to be brought on by an underlying ailment or exposure. The primary causes of vasculitis are systemic illnesses (e.g., rheumatoid arthritis), cancer, drug exposure, and infection. Examples of secondary vasculitis include:

• Rheumatoid vasculitis
• Vasculitis associated with infections such as hepatitis B and C, HIV, infective endocarditis, and tuberculosis
• Vasculitis associated with drugs such as hydralazine and cocaine

Diagnostic tests for vasculitis

Vasculitis is challenging to diagnose, and patients often endure numerous tests and procedures before receiving a definitive diagnosis. The diagnosis of vasculitis typically requires a biopsy of an involved organ, such as the skin, kidney, lung, nerve, or temporal artery. This allows doctors to examine the inflammatory immune cells in the blood vessel wall under a microscope. Here are some of the diagnostic tests and procedures used to confirm vasculitis:

• Skin Biopsy: This is one of the least invasive procedures, performed under local anaesthesia. It involves taking a small piece of skin, usually from the deep dermis, to examine for signs of inflammation or vasculitis.
• Kidney Biopsy: A kidney biopsy is performed if there is evidence of kidney involvement, such as the presence of red blood cells or protein in the urine. This procedure is done under local anaesthesia, and patients are monitored for 24 hours after the biopsy due to the risk of bleeding.
• Sural Nerve Biopsy: The sural nerve is a sensory nerve in the foot. A small piece of the nerve and adjacent muscle is removed under local anaesthesia. This procedure may cause some residual numbness in the foot but usually doesn't affect strength.
• Temporal Artery Biopsy: This biopsy is performed to diagnose Giant Cell Arteritis (GCA) or Temporal Arteritis. The procedure involves making a small incision just above the hairline to access the temporal artery, which runs up the temples. Both temporal arteries may be biopsied to increase diagnostic yield.
• Lung Biopsy: A lung biopsy can be done either as an open lung biopsy or a less invasive thoracoscopic lung biopsy. It is often necessary to diagnose vasculitis involving the lungs, such as granulomatosis with polyangiitis.
• Brain Biopsy: A brain biopsy is often required to confirm Central Nervous System (CNS) vasculitis. It is usually performed on the non-dominant side of the brain and may include a biopsy of the meninges (the brain's covering).
• Angiogram/Angiography: Angiography is particularly useful in diagnosing Polyarteritis Nodosa (PAN). It involves inserting a catheter into a large artery in the leg and advancing it into the aorta to inject radiographic dye into blood vessels. The detection of aneurysms is diagnostic of PAN.
• Central Nervous System Angiogram: This procedure is similar to an abdominal angiogram but involves advancing the catheter up to the large vessels supplying the head and neck. CNS vasculitis is characterised by "beading" (dilated areas alternating with narrowed blood vessels) on angiography.
• Other Useful Tests:

• Erythrocyte Sedimentation Rate (ESR): An old but useful test to assess inflammation.
• C-Reactive Protein (CRP): A protein produced by the liver in response to inflammation.
• Urinalysis: Checking for indicators of inflammation in the urine, such as protein, red blood cells, and clumps of red blood cells, can help determine kidney involvement.
• CT Scan: A cross-sectional radiology test that provides a non-invasive view of the patient's anatomy.
• MRI/MRA: MRI allows for visualisation of the vessel wall and can be useful for diagnosing and following systemic vasculitis, especially large vessel vasculitis.
• ANCA Tests: ANCA (anti-neutrophil cytoplasmic antibodies) are found in the blood of patients with several types of vasculitis, including Wegener's Granulomatosis and Microscopic Polyangiitis.

Treatment options for vasculitis

For mild cases of vasculitis, over-the-counter pain medications can be used to manage symptoms. More severe cases may require prescription medications, such as anti-inflammatory drugs, corticosteroids, immunomodulators, immunosuppressants, or monoclonal antibodies.

Anti-inflammatory Medicine

Non-steroidal anti-inflammatory drugs (NSAIDs) can help reduce pain and lower the body's infection-fighting activity. However, one possible side effect is increased bleeding, so doctors may perform liver function and blood tests before prescribing this medication.

Corticosteroids

Corticosteroids work by lowering the activity of the body's defence system in the blood vessels. They are often required to be taken for months or even years in some types of vasculitis. Common side effects include reduced bone density, increased blood sugar and blood pressure, and thinning of the skin.

Immunomodulators

Immunomodulators reduce the defence system's activity, which helps decrease inflammation and its associated symptoms. However, they may cause gastrointestinal tract problems.

Immunosuppressive Medicine

These medications suppress or weaken the body's ability to fight germs and sickness. They are often used to reduce the dosage and long-term risks associated with glucocorticoids. Possible side effects include an increased risk of infection and birth defects. Examples include rituximab and cyclophosphamide.

Monoclonal Antibodies

Monoclonal antibodies suppress the body's natural defence system. Possible side effects may include fever-like symptoms, stomach pain, and allergic reactions.

Procedures and Surgery

In some cases, procedures or surgery may be required to treat vasculitis. Plasmapheresis is a procedure where blood plasma is removed and then replaced with donor plasma or saline to reduce plasma antibody levels. Vascular bypass surgery may also be performed in severe cases to restore blood flow by redirecting it around a damaged vessel. Surgery is generally a rare treatment option for vasculitis.

Frequently asked questions