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

• Granulomatous Angiitis of the Central Nervous System
• PACNS (Primary Angiitis of the Central Nervous System)
• Primary Central Nervous System Vasculitis (PCNSV)

What is Primary Angiitis of the Central Nervous System? (Definition/Background Information)

• Primary Angiitis of the Central Nervous System (PACNS) is a form of CNS vasculitis that affects the central nervous system (CNS) comprising the brain and spinal cord. The term ‘primary’ is used to indicate that PACNS develops in the absence of an underlying systemic condition, such as an autoimmune disease or infection
• Vasculitis constitute a group of disorders that result in the inflammation of the blood vessels. 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. A secondary form of CNS vasculitis is seen in the presence of certain conditions that affect the whole body
• Primary Angiitis of the Central Nervous System is observed in middle-aged adults and typically involves only the central nervous system. The affected individuals may present severe headaches, vision impairment, muscle weakness, and seizures, depending on the severity of the condition
• It is generally difficult to diagnose Primary Angiitis of the Central Nervous System due to its extreme rarity and the presence of certain non-specific symptoms. 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 Primary Angiitis of the Central Nervous System, usually with high-dose steroids and immunosuppressants, can lead to a good prognosis. However, late recognition and delayed management of the condition can severely worsen the outcomes, due to complications such as brain dysfunction and strokes

Who gets Primary Angiitis of the Central Nervous System? (Age and Sex Distribution)

• Primary Angiitis of the Central Nervous System can be generally seen across a wide age group affecting both males and females; both adults and children are affected
• However, the peak age of diagnosis is around 50 years and males are generally affected more than females; according to some studies, a 2:1 male to female ratio is noted
• It is observed worldwide and can affect people of all racial and ethnic backgrounds

What are the Risk Factors for Primary Angiitis of the Central Nervous System? (Predisposing Factors)

• Currently, no risk factors have been clearly established for Primary Angiitis of the Central Nervous System
• The condition is not associated with any infectious or non-infectious conditions (such as autoimmune disorders)

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 Primary Angiitis of the Central Nervous System? (Etiology)

• Primary Angiitis of the Central Nervous System is described as an inflammation of the small and medium-sized blood vessels affecting the central nervous system (CNS)
• In this condition, the inflammation of the blood vessels is confined to the brain and spinal cord. No underlying or coexisting systemic disorders are identified

What are the Signs and Symptoms of Primary Angiitis of the Central Nervous System?

The severity of signs and symptoms of Primary Angiitis of the Central Nervous System (PACNS) 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 PACNS are associated with the brain and spinal cord.

The common signs and symptoms (constitutional symptoms) of Primary Angiitis of the Central Nervous System 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

How is Primary Angiitis of the Central Nervous System Diagnosed?

According to medical literature, it is usually difficult to establish a diagnosis of Primary Angiitis of the Central Nervous System (PACNS). Since the condition is rare, it is often misdiagnosed; or, can cause significant delays towards establishing an accurate diagnosis. Hence, in order to arrive at a diagnosis of PACNS, all ‘potential’ underlying disorders may have to be ruled out

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
  • 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
• A differential diagnosis is usually considered to eliminate a group of conditions called reversible cerebral vasoconstriction syndrome (RCVS) that presents similar signs and symptoms to CNS vasculitis

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 Primary Angiitis of the Central Nervous System?

The complications of Primary Angiitis of the Central Nervous System that develop depend on the 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; it may be noted in about 20% of the individuals
• 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

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 Primary Angiitis of the Central Nervous System 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 Primary Angiitis of the Central Nervous System Treated?

The treatment of Primary Angiitis of the Central Nervous System 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

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 for the condition may include:

• 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
• 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 Primary Angiitis of the Central Nervous System be Prevented?

Currently, there are no methods available to prevent Primary Angiitis of the Central Nervous System. An early diagnosis and prompt treatment can help decrease the burden of the condition.

• 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 Primary Angiitis of the Central Nervous System? (Outcomes/Resolutions)

• The prognosis of Primary Angiitis of the Central Nervous System (PACNS) 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
  • 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 PACNS can be either short-term or long-term, leading to the development of acute or chronic conditions, respectively. In some affected individuals, the condition is known to relapse after a period of remission
• With early diagnosis and treatment, the prognosis is good for many individuals. If left untreated, the condition can potentially cause significant tissue injury, organ failure, or even death

Even with treatment, PACNS 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 Primary Angiitis of the Central Nervous System:

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

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