What is Vasculitis?

 

 

https://www.nhlbi.nih.gov/health-topics/vasculitis

Vasculitis

Also known as Angiitis, Arteritis

Vasculitis includes a group of rare conditions that can occur when inflammation affects the walls of your blood vessels. Inflammation is your body’s response to tissue injury. Autoimmune disorders, infections, and trauma are some examples of potential triggers of inflammation in the blood vessels. Inflammation in the blood vessels can lead to serious problems, including organ damage and aneurysms.

There are many different types of vasculitis. Vasculitis can affect any of the blood vessels in the body. With vasculitis, you may experience general signs and symptoms, such as fever, weight loss, fatigue, pain, and rash. You may have other symptoms depending on the part of the body that is affected and the severity of the vasculitis. If your doctor diagnoses you with vasculitis, medicine can help improve your symptoms and help you avoid flares and complications.

Explore this Health Topic to learn more about vasculitis, our role in research and clinical trials to improve health, and where to find more information.

 

 Types

There are many different types of vasculitis that affect different blood vessels throughout the body.

Types of vasculitis include the following.

• Anti-glomerular basement membrane disease affects blood vessels in the lungs and kidneys.
• Behçet’s disease can cause damage to many organs.
• Buerger’s disease, also known as thromboangiitis obliterans, usually affects blood flow to the arms and legs.
• Central nervous system vasculitis affects the blood vessels in the central nervous system, or the brain and spinal cord. It is also called primary angiitis of the central nervous system. This type of vasculitis may also occur as the result of another type of vasculitis.
• Cogan’s syndrome is an autoimmune disorder associated with a particular type of vasculitis that affects the whole body.
• Cryoglobulinemic vasculitis affects the small blood vessels. It prevents proper blood flow and causes pain and damage to the skin, joints, peripheral nerves, kidneys, and liver.
• Eosinophilic granulomatosis with polyangiitis, also known as Churg-Strauss syndrome, often affects the respiratory tract.
• Giant cell arteritis mostly affects the aorta or its major branches. The condition often affects the temporal artery in the head.
• Granulomatosis with polyangiitis usually affects the upper respiratory tract, lungs, and kidneys.
• Hypersensitivity vasculitis affects the skin. This condition also is known as allergic vasculitis, cutaneous vasculitis, or leukocytoclastic vasculitis.
• Hypocomplementemic urticarial vasculitis is associated with a certain type of antibody.
• Immunoglobulin A (IgA) vasculitis, also known as Henoch-Schönlein purpura, is one of the most common types of vasculitis in children but can also affect adults. It develops when IgA, which is a type of antibody that usually helps defend the body against infections, builds up in blood vessels in the skin, joints, intestines, and kidneys.
• Kawasaki disease is a rare childhood disease that develops when the walls of the blood vessels throughout the body become inflamed. Kawasaki disease is also known as mucocutaneous lymph node syndrome.
• Microscopic polyangiitis affects small blood vessels, often including those in the kidneys and lungs.
• Polyarteritis nodosa causes swelling and damage most often to medium-sized arteries. This type of vasculitis may cause muscle pain or gastrointestinal symptoms.
• Takayasu arteritis most often affects the aorta and its branches. The condition can also affect medium-sized arteries.

 

 

 https://www.allergy.org.au/patients/autoimmunity/vasculitis-disorders

Vasculitis Disorders

ASCIA PCC Vasculitis disorders 2019162.14 KB

Vasculitis disorders result from inflamed of blood vessels. They are relatively rare and can affect people of all ages. Granulomatosis with polyangiitis (also known as Wegener’s granulomatosis) is the most common form of vasculitis, and it affects around five in a million people.

Vasculitis disorders cause a wide range of symptoms, that can affect the skin and internal organs. Treatment length varies, and some people need to use medications for long periods of time.

What is vasculitis?

Vasculitis disorders result from inflammation of blood vessels, including arteries, arterioles, veins, venules and capillaries. The inflammation causes a narrowing of blood vessels, which can result in blood flow obstruction (ischaemia).  This may lead to tissue damage (necrosis) and blood clots (thrombosis). 

What causes vasculitis?

There are three main underlying causes of vasculitis disorders:

• Autoimmunity.
• Allergy or hypersensitivity to medications, toxins or other inhaled environmental irritants (where removing the medication, toxin or irritant usually stops symptoms).
• Viral or parasite Infections.

Allergy, hypersensitivity and infections should be ruled out before autoimmunity is considered as the cause of a vasculitis disorder.  

Autoimmune related vasculitis

The main role of the immune system is to defend against infections (such as bacteria, moulds and viruses) and other invaders (such as cancer cells), whilst protecting the body’s own cells. Autoimmunity occurs when the body doesn’t recognise its own cells and attacks them.

Antibodies produced by the immune system in vasculitis disorders cause inflammation in blood vessels that can lead to problems. Complications depend on which blood vessels, organs and other systems are affected.   

Vasculitis disorders may also occur in people with other autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus (SLE) and dermatomyositis. 

Diagnosis 

Due to the wide range of signs, symptoms and body systems involved, an extensive history and physical examination is needed to diagnose the type of vasculitis disorder. Blood tests are taken, and in some cases an x-ray or biopsy may be required. An exact diagnosis is needed to provide the right treatment.

Treatment

When vasculitis is due to an autoimmune disorder, immunosuppressive drugs are usually used.

Plasmapheresis (a procedure that filters the offending autoantibodies out of the blood plasma and returns the filtered blood back to the patient), may be used in serious cases that do not respond to other treatment.

Signs and symptoms

General signs and symptoms of vasculitis disorders include:

• Fever.
• Loss of appetite and weight loss.
• Fatigue, weakness and lethargy.
• General aches and pains.

Specific signs and symptoms of vasculitis disorders include:

• Skin may have purple or red spots or bumps, clusters of small dots, splotches, bruises, urticaria (hives), itch.
• Joints may have pain, arthritis in one or more joints.
• Lungs display a shortness of breath, coughing up blood, signs that suggest pneumonia.
• Gastrointestinal tract as indicated by mouth ulcers (sores), or stomach pain. In severe cases blood flow to the intestines can be blocked.
• Sinuses, nose, throat, ears where chronic (ongoing) sinus or middle ear infections, ulcers in the nose, and hearing loss are evident.
• Eyes may be red, itchy and burning, increased sensitivity to light, blurred vision. 
• Brain may experience headaches, changes in mental function, stroke-like symptoms such as muscle weakness and paralysis.
• Nerves resulting in numbness, tingling, and weakness in various body parts, loss of feeling or strength in hands and feet, shooting pains in arms and legs.

Examples of vasculitis disorders (small blood vessels)

• Granulomatosis with polyangiitis affects sinuses, lungs, kidneys, and skin.
• Eosinophilic granulomatosis with polyangiitis affects the lungs, skin, and nerves.
• Cryoglobulinaemia affects skin, kidneys, and nerves.
• Goodpasture’s syndrome affects lungs and kidneys.
• Henoch-Schonlein purpura affects skin, joints, kidneys, and gut.
• Microscopic polyangitis affects skin, kidneys, and nerves.

Examples of vasculitis disorders (medium blood vessels)

• Behcet’s disease affects mucous membranes, skin, and eyes.
• Central nervous system vasculitis affects the brain.
• Kawasaki syndrome affects skin, mucous membranes, lymph nodes, and blood vessels.
• Polyarteritis nodosa affects arteries, kidneys, gut, nerves, and skin.

Examples of vasculitis disorders (large blood vessels)

• Giant cell (temporal) arteritis affects arteries of the head and neck.
• Takayasu arteritis affects arteries of the head and neck.
• Polymyalgia rheumatic produces inflammation and swelling in joint and muscles.

© ASCIA 2019

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