What are the other Names for this Condition? (Also known as/Synonyms)

• Central Nervous System Vasculitis
• Cerebral Vasculitis

What is CNS Vasculitis? (Definition/Background Information)

• Vasculitis constitute a group of disorders that result in the inflammation of the blood vessels. It can cause damage to various body organs depending on the region affected, due to decreased blood and oxygen supply to the body region
• Central Nervous System Vasculitis (or CNS Vasculitis) is a very rare condition that affects the small and medium-sized arteries and veins. It is classified either as the primary form or the secondary form
  • Primary angiitis of the central nervous system (PACNS): It is the primary form of CNS Vasculitis that is not seen in the background of any systemic condition
  • Secondary CNS Vasculitis: It is the secondary form of CNS Vasculitis that is seen in the presence of certain conditions that affect the whole body
• CNS Vasculitis affects the central nervous system (CNS) comprising the brain and spinal cord. It is also known as Cerebral Vasculitis. Individuals may present severe headaches, vision impairment, muscle weakness, and seizures, depending on the severity of the condition. Apart from this, the signs and symptoms of the coexisting condition, if any present, may be noted
• The risk factors for the primary form of CNS Vasculitis are unknown. Autoimmune disorders associated with the secondary form of CNS Vasculitis include Sjogren’s syndrome and systemic lupus erythematosus. Sometimes, it is also seen with other forms of systemic vasculitis such as Behcet’s syndrome and microscopic polyangiitis (MPA)
• It is generally difficult to diagnose CNS Vasculitis due to the presence of certain non-specific symptoms and coexisting conditions. A wide variety of tests and exams may be performed including blood and urine tests, radiological studies, neurological assessment, and a brain tissue biopsy, if necessary
• An early diagnosis and appropriate treatment of CNS Vasculitis, usually with high-dose steroids and immunosuppressants, can lead to a good prognosis. However, a late recognition and delayed management of the condition can severely worsen the outcomes, due to complications such as brain dysfunction and strokes

Who gets CNS Vasculitis? (Age and Sex Distribution)

• CNS Vasculitis can be generally seen across a wide age group affecting both males and females; both adults and children are affected
• It is observed worldwide and can affect people of all racial and ethnic backgrounds

What are the Risk Factors for CNS Vasculitis? (Predisposing Factors)

• Currently, no risk factors have been clearly established for the primary form of CNS Vasculitis. It is not associated with any infectious or non-infectious conditions
• Secondary CNS Vasculitis is associated with certain infectious and non-infectious conditions (such as autoimmune disorders). It is also observed in the background of other subtypes of vasculitis

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of CNS Vasculitis? (Etiology)

CNS Vasculitis is a rare and serious disorder in which there is inflammation of the small and medium-sized blood vessel that supplies blood to the brain and spinal cord. The condition can result in severe brain dysfunction.

• No underlying systemic disorders are identified in the primary form of CNS Vasculitis (i.e., primary angiitis of the central nervous system)
• Secondary CNS Vasculitis usually occurs in association with other systemic autoimmune disorders. In some cases, it can be triggered by a viral or bacterial infection

In many cases, CNS Vasculitis is believed to be the result of a hypersensitivity reaction to certain stimuli triggering an abnormal immune reaction. Such stimuli could be in the form of inflammatory disorders, infections, or various other factors.

What are the Signs and Symptoms of CNS Vasculitis?

The severity of signs and symptoms of CNS Vasculitis may vary from one individual to another. In some, it may be mild, resulting in a delayed diagnosis of the condition. In other individuals, it may be severe. The signs and symptoms of CNS Vasculitis are mostly associated with the brain and spinal cord and also on the coexisting autoimmune, vascular, or infectious condition.

The common signs and symptoms (constitutional symptoms) of CNS Vasculitis include:

• Fever
• Fatigue and tiredness
• Loss of appetite
• Weight loss
• Weakness
• Skin rashes
• Joint pain

It is important to note that the combination of signs and symptoms varies from individual to individual. The signs and symptoms related to the central nervous system (CNS) may include:

• Headaches that may be of sudden onset; persistent headaches
• Nausea and vomiting
• Damage to peripheral nerves
• Numbness and tingling sensation
• Muscle weakness; loss of strength
• Rigidness of muscles
• Neck pain and/or back pain that may involve the lower part of the body
• Visual impairment such as blurred vision, double vision, or poor eyesight
• Speech impairment; aphasia (when one’s ability to communicate is affected)
• Hearing loss
• Insomnia or loss of sleep, or excessive sleepiness (usually during daytime)
• Tremors
• Seizures
• Lack of coordination; unsteadiness
• Dizziness and vertigo
• Loss of balance
• Confusion
• Fainting
• Change in behavior
• Mental impairment
• Forgetfulness and memory loss

The specific signs and symptoms of the underlying condition may be additionally noted. In some individuals, CNS Vasculitis may be mistaken for multiple sclerosis (MS), a neurodegenerative condition involving the central nervous system.

How is CNS Vasculitis Diagnosed?

According to medical literature, it is usually difficult to establish a diagnosis of CNS Vasculitis. Since the condition is rare, it is often misdiagnosed; or, it may lead to significant delays towards establishing a correct diagnosis. Contributing to a delayed diagnosis is the fact that the signs and symptoms related to CNS Vasculitis overlap with other common conditions affecting the brain and spinal cord.

The diagnosis may involve the following tests and examinations:

• Complete physical examination and a thorough medical history
• Assessment of the presenting signs and symptoms

Tests and procedures based on the different body part/organ or system that is affected may include any of the following.

• Blood and urine tests related to overall inflammatory and autoimmune activity:
  • Complete blood count (CBC) with differential: This measures the red blood cell count and hemoglobin levels in blood
  • Erythrocyte sedimentation rate (ESR): ESR is a blood test that can help the healthcare provider identify any inflammatory activity within the body
  • C-reactive protein (CRP) test: This blood test measures the amount of a protein, called C-reactive protein, in blood. It also provides information on the inflammation condition of the body
  • Anti-nuclear antibody (ANA) test: ANA test identifies the antinuclear antibodies within the blood
  • Basic metabolic panel tests
  • Anti-streptolysin O (ASO) titer test, if a streptococcal infection is the suspected trigger
  • Specific antibodies test for systemic lupus erythematosus (SLE) and Sjogren syndrome
  • Protein electrophoresis and immunofixation electrophoresis tests
  • Complement blood test
  • Cryoglobulin blood test
  • HIV antibody blood test
  • Urine tests for protein and presence of blood
  • Urinalysis

Tests and procedures related to the brain and spinal cord (central nervous system):

• Neurological, motor skills, and cognitive assessment: Checking intellectual ability, memory, mental health and function, language skills, judgment and reasoning, coordination and balance, reflexes, sensory perceptions (space, sight, hearing, touch)
• Electroencephalography (EEG)
• Electromyography with nerve conductivity tests
• Imaging studies that may be performed include:
  • X-ray of head and neck and/or vertebral column
  • CT scan of the head and neck region and/or vertebral column
  • MRI scan of the central nervous system (brain and spine)
  • Cerebral angiographic studies
• Cerebrospinal fluid analysis, where a spinal tap test may be performed
• Tissue biopsy of the affected blood vessel or brain:
  • A tissue biopsy is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis
  • Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation
  • Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing, and very rarely, electron microscopic studies to assist in the diagnosis
• Other diagnostic examinations may be performed based on the affected region/part of the body and the nature of the coexisting condition

Differential diagnosis: It is essential to exclude a condition called reversible cerebral vasoconstriction syndrome (RCVS). RCVS may have similar signs and symptoms to CNS Vasculitis. However, in many cases, such signs and symptoms may be temporary, since the condition is caused by a temporary spasm of the blood vessel. The treatment and prognosis of the two conditions are also significantly different.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of CNS Vasculitis?

The complications of CNS Vasculitis that develop depend on the underlying cause, if any identified, the organs affected, severity of the signs and symptoms, and one’s response to treatment.

Complications associated with the brain and spinal cord (central nervous system) may include:

• Severe and persistent headaches
• Encephalopathy or swollen brain
• Transient ischemic attack (TIA) and brain stroke
• Increased risk for fall injuries due to balance issues
• Unable to stand or walk
• Paralysis, usually affecting one side of the body
• Coma
• Vision loss
• Reduced performance at school or work
• Severely affected quality of life due to mental health issues

Additionally, the complications of the associated conditions, such as infections, autoimmune disorders, or other vasculitis forms, may be noted.

Treatment complications: Some complications are known to take place with treatment; in some cases, due to treatment also. Immunosuppressive therapy that is used to treat CNS Vasculitis may lead to certain complications that include:

• Low blood pressure
• Hyperkalemia or high levels of potassium in blood
• Tremors
• Diabetes mellitus
• Osteoporosis causing decreased bone density
• Decreased muscle function
• Long-term kidney dysfunction
• Elevated risk for malignancy
• Decrease in body immunity to fight infections

How is CNS Vasculitis Treated?

The treatment of CNS Vasculitis depends upon a variety of factors including:

• Severity of the signs and symptoms
• Regions of the body affected and the level of functioning of the affected organs
• Overall health status of the individual and his/her age
• Nature of the underlying cause or associated condition, if any observed

The main treatment of CNS Vasculitis includes treating the associated/underlying condition. Often, a multidisciplinary team of specialists that include general practitioners, rheumatologists, neurologists, pulmonologists, cardiovascular experts, nephrologists, dermatologists, ophthalmologists, among others, may be involved in the treatment.

The treatment methods may include:

• Most cases are thought to arise as a result of hypersensitivity to an offending stimulus, hence removal of the stimulus is often sufficient treatment for the illness
• Corticosteroid therapy, usually using high-dose prednisone
• Immunosuppressive medications such as cyclophosphamide, azathioprine, or mycophenolate mofetil
• Biologic agents using specific monoclonal antibodies to reduce inflammation
• In cases with severe symptoms, or when the affected individuals do not respond to the above-mentioned treatments, plasma exchange (plasmapheresis) or intravenous immunoglobulin (IVIG) therapy may be considered
• Rarely, vascular surgery, such as vascular bypass procedures, vascular stent placements, and removal of blood clots (thrombectomy), are found to be beneficial
• Physical therapy including hearing and speech therapy with the help of speech-language pathologists
• Occupational therapy

The treatment also depends on whether there are recurring episodes of the condition. The goal of the treatment is to control the inflammation in order to achieve remission (a state where no signs and symptoms are noted) and maintenance therapy to prevent episodes of relapses.

How can CNS Vasculitis be Prevented?

Currently, there are no methods available to prevent CNS Vasculitis. An early diagnosis and prompt treatment can help decrease the burden of the condition.

• Undertaking early and appropriate treatment of the underlying infection or inflammatory disorder may help in lowering one’s risk for the condition
• Managing the underlying infection or inflammatory condition that triggered the initial episode helps in preventing subsequent episodes of CNS Vasculitis
• Maintaining a healthy lifestyle with good eating habits, adequate physical activities, and avoidance of smoking or drinking is beneficial
• Ensuring that individuals, especially children, are up-to-date on their vaccinations is important
• Patients are required to be vigilant during the remission period and report any new symptoms promptly to the healthcare provider

What is the Prognosis of CNS Vasculitis? (Outcomes/Resolutions)

• The prognosis of CNS Vasculitis depends upon a variety of factors including the:
  • Severity of the condition; degree of damage to the vital organs and body systems
  • Age and overall health of the individual
  • One’s response to treatment: Individuals who respond to treatment usually have a better prognosis than those who do not respond well to treatment
  • The underlying condition that triggered CNS Vasculitis, if any present
  • Whether it is an initial episode or a recurring episode
• Individuals with extensive condition affecting several regions of the body have poorer prognosis, than individuals in whom the condition is confined to certain part(s) of the body
• The inflammation in CNS Vasculitis can be either short-term or long-term, leading to the development of acute or chronic conditions, respectively. It is reported that about 25% of the affected individuals will have relapses
• With early diagnosis and removal of the offending agent, the prognosis is good for many individuals. If left untreated, the condition can potentially cause significant tissue injury, organ failure, permanent damage to the neurological system, or even death

Even with treatment, CNS Vasculitis can relapse. If blood in cough or blood in urine is observed, then it is extremely important to contact the healthcare provider right away.

Additional and Relevant Useful Information for CNS Vasculitis:

The following article link will help you understand other Vein & Vascular Health:

https://www.dovemed.com/health-topics/vein-and-vascular-center/