Bay Biosciences provides high quality, clinical grade, biopsy tissue samples, FFPE tissue blocks with matched cryogenically preserved sera (serum), plasma, peripheral blood mononuclear cells (PBMC) bio-fluid samples from patients diagnosed with vasculitis.
The sera (serum), plasma and PBMC biofluid specimens are processed from vasculitis patient’s peripheral whole-blood using customized collection and processing protocols.
The vasculitis sera (serum), plasma and peripheral blood mononuclear cells (PBMC) biofluids are processed from patient’s peripheral whole-blood using customized collection and processing protocols.
Vasculitis Overview
Vasculitis is a general term for several conditions that cause inflammation in the blood vessels. It’s also called angiitis or arteritis. It can make the blood vessels weak, stretched, bigger, or narrower. They might close entirely.
This condition can affect patients of any age. Some forms affect blood vessels that go to or supply specific organs like the skin, eyes or brain. Other kinds might involve many organ systems at the same time. Some of these general forms could be mild and not need treatment. Others may be severe.
Vasculitis can be acute and short-term or chronic and long-term. In some cases, the organs in the body may be affected, especially if they do not receive enough nutrient and oxygen-rich blood, resulting in organ damage, and sometimes death.
There are various different causes and types of vasculitis, which lead to specific symptoms and management.
Each of the vasculitis diseases is defined by certain patterns of distribution of blood vessel involvement, particular organ involvement, and laboratory test abnormalities. As a group, these diseases are referred to as vasculitides.
The word vasculitis is derived from the Latin “vasculum”, vessel + “- itis,” inflammation. Another term for vasculitis is angiitis. When arteries are the inflamed blood vessels, the condition is also referred to as arteritis. When the veins are inflamed, it is referred to as venulitis.
Types of Vasculitis
Usually, vasculitis is divided into three different types small, medium and large based on the size of the blood vessels involved:
- Small: This type of vasculitis includes Behcet’s syndrome, Churg-Strauss syndrome, cutaneous vasculitis, Henoch-Schonlein purpura, microscopic polyangiitis, granulomatosis with polyangiitis, golfer’s vasculitis, and cryoglobulinemia.
- Medium: Examples are Buerger’s disease, cutaneous vasculitis, Kawasaki disease, and polyarteritis nodosa.
- Large: This includes conditions like polymyalgia rheumatica, Takayasu’s arteritis, and temporal arteritis.
Following are some of the common types of vasculitis:
- Giant cell arteritis leads to the inflammation of medium and large arteries, commonly those in the head and neck. Common symptoms of this type are aching around the temples, muscle pain in the jaw and loss of vision. Elderly people are more susceptible to the condition, although the cause is unclear.
- Polymyalgia rheumatic leads to inflammation, pain and stiffness in the shoulder, neck and hip muscles. Women are twice as likely as men to experience this, particularly elderly women.
- Takayasu arteritis affects the coronary artery, which gradually becomes narrower and reduces blood supply to the body.
- Polyarteritis nodosa involves damaged and inflamed small and medium arteries. The blood vessels that supply blood to the gastrointestinal tract, kidneys and nerves are often affected to a greater extent and can cause internal bleeding in these areas. It can also block an artery entirely, causing potentially serious outcomes.
- Kawasaki disease affects the small and medium arteries of young children. Characteristic symptoms include continued fever, rash, swollen glands, red eyes, lips, tongue or mouth. As it can also affect coronary arteries, heart complications may arise.
- Granulomatosis with polyangiitis is also known as Wegener’s granulomatosis and affects the vessels supplying blood to the nose, sinuses, ears, kidneys and lungs. It is potentially fatal in the case of tissue damage and kidney impairment and prompt medical treatment is, therefore, essential. Other symptoms may include fever, sinusitis and shortness of breath.
- Eosinophilic granulomatosis with polyangiitis is also known as Churg-Strauss syndrome and causes symptoms of allergic sinusitis and asthma. It usually affects adults and includes various symptoms such as fatigue, fever, muscle ache and weakness.
- Microscopic polyangiitis commonly affects blood vessels in the lungs, kidneys and nervous system. Common symptoms include hypertension, kidney impairment, anemia and shortness of breath.
- Henoch-Schönlein purpura usually affects children and is characterized by a skin rash and pain of the stomach and joints. It often occurs after a recent infection and is self-limiting, rarely leading to serious outcomes.
In addition, there are many other types of vasculitis that are less commonly seen in practice.
Causes of Vasculitis
Exact causes of vasculitis diseases are usually not known. However, immune system abnormality and inflammation of blood vessels are common features. Each form of vasculitis has its own characteristic pattern of symptoms, much of which depends on what particular organs are affected.
It might be linked to an allergic reaction to a medication. You can also get it after having an infection that sets off an unusual response in your immune system, damaging your blood vessels.
Vasculitis also may be tied to immune system diseases that you’ve had for months or years, such as rheumatoid arthritis, lupus, or Sjögren’s Syndrome.
Vasculitis can also accompany the following conditions:
- Infections (such as hepatitis B),
- Exposure to chemicals (such as amphetamines and cocaine),
- Medications,
- Cancers (such as lymphomas and multiple myeloma (MM)), and
- Rheumatic diseases (such as rheumatoid arthritis and systemic Lupus Erythematosus (SLE)
Signs and Symptoms of Vasculitis
General signs and symptoms of most types of vasculitis include:
- Fever
- General aches and pain
- Headache
- Fatigue
- Unexplained Weight Loss
Other signs and symptoms of vasculitis are related to the parts of the body affected, including:
- Digestive system: If the patient’s stomach or intestines are affected, the patient may experience pain after eating. Ulcers and perforations are possible and may result in blood in the stools.
- Ears: Dizziness ringing in the ears and abrupt hearing loss may occur.
- Eyes: Vasculitis can make your eyes look red and itch or burn. Giant cell arteritis can cause double vision and temporary or permanent blindness in one or both eyes. This is sometimes the first sign of vasculitis.
- Hands or feet: Some types of vasculitis can cause numbness or weakness in a hand or foot. The palms of the hands and soles of the feet might swell or harden.
- Lungs: You may develop shortness of breath or even cough up blood if vasculitis affects the lungs.
- Skin: Bleeding under the skin can show up as red spots. Vasculitis can also cause lumps or open sores on the skin.
The following are signs and symptoms of specific vasculitis conditions:
Behcet’s Disease
Symptoms vary and can disappear and return. The affected areas include the following:
- Mouth: Painful sores, like canker sores. They start as raised, round lesions in the mouth and become painful ulcers.
- Skin: Some patients may develop acne-like sores while others may have red, raised, and tender nodules, especially on the legs.
- Genitals: Most commonly on the scrotum in males and the vulva in females. They appear as red, round, ulcerated lesions.
- Eyes: Inflammation of the eye, called uveitis.
- Joints: Typically swelling and pain in the knees, and sometimes the ankles, elbows, or wrists.
- Blood vessels: Inflammation of the veins and large arteries causing painful and swollen limbs.
- Gastrointestinal: Abdominal pain, diarrhea. Possible bleeding in the digestive system.
- Brain: Inflammation in the brain and nervous system can result in headaches, fever, poor balance, and disorientation.
Buerger’s Disease
Buerger’s disease affects the arteries and veins in the arms and legs. The blood vessels swell and become blocked with blood clots, called thrombi, eventually damaging or destroying skin tissues.
Sometimes, this can lead to infection and gangrene. Risk of Buerger’s disease is linked to regular smoking.
Initial symptoms often include pain in the feet and hands during exercise caused by insufficient blood flow. Sometimes this pain may also be present at rest. Usually, the pain starts in the extremities but may radiate to other parts of the body. A patient may also experience:
- Affected areas may be extremely painful
- Fingers, toes, hands, and feet turn white in the cold
- Gangrene of fingers and toes
- Numbness in the limbs
- Skin ulcerations
- Tingling in the limbs
Central Nervous System Vasculitis
This is vasculitis that involves the brain and spinal cord. Signs and symptoms may include:
- Dysphasia, coma
- Depression
- General confusion
- Headaches
- Mental changes
- Myelopathy, which is a disorder of the spinal cord
- Paralysis or muscle weakness
- Seizures
- Visual problems
Churg-Strauss Syndrome
This rare syndrome is an inflammation of small arteries and veins in people with a history of allergy or asthma.
Its main feature is asthma, which may begin long before the onset of vasculitis. Early signs and symptoms may include:
- Allergic rhinitis
- Nasal polyps
In the next phase, the person typically has eosinophilia, a condition when there are too many eosinophils, a type of white blood cell.
The third phase is a vasculitis usually involving the skin, lungs, nerves, kidneys, and other organs.
There is frequent devastation of the nerves, called mononeuritis multiplex, which causes severe tingling, numbness, muscle wasting in hands and feet, and shooting pains.
Cryoglobulinemia
This condition is linked to hepatitis C infections. It causes the blood to become abnormally thick with inflammation of blood vessels.
Symptoms vary according to the type and which organs are affected. Typically, signs and symptoms include:
- Breathing problems
- Fatigue
- Fingers, toes, hands, and feet turning white in the cold
- Glomerulonephritis affecting the kidneys
- Joint pain
- Muscle aches
- Purpura, or purple spots and patches on the skin, organs, and mucous membranes
- Skin ulceration
Giant Cell Arteritis
This is inflammation of the walls of the arteries. The most common symptoms are:
- Blurred vision
- Headache
- Pain in the jaw after chewing
- Shoulder pain
- Pain in the hips
Less common symptoms include:
- Cough
- Depression
- Scalp tenderness
- Throat pain
- Tongue pain
- Pain in the arms during exercise
- Weight loss
Henoch-Schönlein Purpura
Inflammation of the tiny blood vessels, called the capillaries, in the skin and frequently in the kidneys, resulting in skin rashes, especially over the buttocks and behind the lower extremities. It is associated with arthritis and sometimes cramping pain in the stomach.
The most common symptoms are:
- A skin rash that looks similar to small bruises, or red spots on the buttocks, elbows, and legs
- Blood in stools if the vessels in the bowel and kidneys are inflamed
- Diarrhea
- Joint pain especially in the knees and ankles
- Stomach pain
- Nausea
- Vomiting
Kawasaki Disease
This is a rare syndrome of unknown origin. It causes inflammation in the walls of arteries throughout the body, including the coronary arteries supplying blood to the heart muscle.
Kawasaki disease causes high fever, reddening of the eyes (conjunctivitis), lips and mucous membrane of the mouth, gingivitis (ulcerative gum disease), swollen neck glands, and a bright red rash on the hands and feet. It affects young children.
Takayasu’s Arteritis
This is a rare type of vasculitis where inflammation damages the aorta, which is the large artery carrying blood from the heart to the rest of the body. It also damages the aorta’s main branches.
A person with Takayasu’s arteritis experiences arm or chest pain, hypertension causing high blood pressure, and they may eventually experience a Stroke or heart failure.
Wegener’s Granulomatosis
Wegner’s granulomatosis causes inflammation and injury to blood vessels and affects several organs including the lungs, kidneys, and upper respiratory tract.
It is a life-threatening disorder that requires long-term immunosuppression.
Some people can die due to the toxicity of the treatment.
Rapidly Progressive Glomerulonephritis
This condition is a syndrome of the kidney that rapidly progresses into acute renal (kidney) failure if untreated.
Diagnosis of Vasculitis
Your doctor will ask about your medical history and do a physical exam. You’ll need tests to look for inflammation or to rule out other conditions.
Laboratory testing of blood or body fluids in a patient with active vasculitis generally indicates inflammation in the body. Depending on the degree of organ involvement, a variety of organ function tests can be abnormal.
The diagnosis of vasculitis is definitively established after a biopsy of involved tissue demonstrates the pattern of blood vessel inflammation. Examples of tissues used for biopsy include skin, sinuses, lung, nerve, and kidneys.
Depending upon the situation, an alternative to biopsy can be an X-ray test of the blood vessels called an angiogram, which can demonstrate characteristic patterns of inflammation in affected blood vessels.
Following are the common tests performed to diagnose vasculitis:
- Blood tests. Certain types of blood cells or antibodies can be signs of vasculitis.
- Urine tests. These check for kidney damage.
- Imaging Tests, X-rays, MRI scans, CT scans, PET scans, and ultrasounds show inflammation in your blood vessels and organs. You might also have an angiogram, in which your doctor injects dye into your bloodstream. It shows up on X-rays to give a better picture of your blood vessels.
- Heart Tests: An echocardiogram tests how well your heart is working
- Biopsy: Your doctor takes a sample of tissue from your blood vessel or organ. A specialist can check it for signs of inflammation or damage.
Treatment of Vasculitis
Treatment for vasculitis depends on several factors, including what type of vasculitis the person has, the severity of their symptoms, their age, and their overall general health.
Sometimes, as may be the case with Henoch-Schonlein purpura, the condition clears up without medical treatment.
The treatment of the various forms of vasculitis is based on the severity of the illness and the organs involved.
- Treatments are generally directed toward stopping the inflammation and suppressing the immune system.
- Typically, cortisone-related medications, such as prednisone, are used.
- Additionally, other immune suppression drugs, such as cyclophosphamide (Cytoxan) and others are considered.
- Additionally, affected organs (such as the heart or lungs) may require specific medical treatment when the disease is active.
The management of vasculitis is an evolving field in medicine. The ideal programs for monitoring and treatment will continue to improve as disease patterns and causes are defined by medical research.
Following are some of the most commonly used medications for vasculitis:
Steroids
These are used to reduce inflammation. Examples include prednisone or methylprednisolone (Medrol). Side effects may be severe if taken over the long-term.
They may include the following:
- Diabetes
- Osteoporosis (thinning of the bones)
- Weight gain
Immune System Medications
If a patient does not respond well to steroid therapy, a doctor may prescribe cytotoxic drugs that stop the immune system cells that cause inflammation.
Examples include the following:
- Azathioprine (Imuran)
- Cyclophosphamide (Cytoxan)
Complications of Vasculitis
Vasculitis can be serious. When your blood vessel becomes weak, it might stretch and bulge (called an aneurysm). It might also burst open, causing bleeding. This can be life-threatening but is very rare.
When your blood vessel becomes inflamed and narrowed, it might partially or totally block the blood flow to that area of your body. If you don’t have enough other blood vessels to supply the area, the tissue will die. This can happen in any part of your body.
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