Vasculitis Types

Aortitis

Aortitis is an umbrella term for inflammation of the aorta.The aorta is the largest artery that carries blood from the heart and distributes it to the body through smaller arteries. The most common causes of aortitis due to the large vessel vasculitis disorders giant cell arteritis (GCA) and Takayasu’s arteritis although may be seen in other rheumatological diseases including systemic lupus erythematosus, Behcets syndrome, sarcoidosis and Cogans syndrome.

Aortitis may also arise due to infections including tuberculosis, salmonella and bacterial infections. Inflammation of the aorta may lead to blockage, or weakening of the vessel wall leading to aneurysms. It has the potential to cause other severe complications including kidney failure, stroke, heart failure, and aortic rupture, which can be fatal.

Who Gets Aortitis?

The prevalence of aortitis is not well documented, current estimates of incidence is approximately 1-3 per million people each year. It affects both males and females equally and at any age.

Symptoms

Symptoms vary according to the cause and the site and may include:

Back pain
Abdominal pain
Shortness of breath
Swelling (Edema) of the legs
Fever
Night Sweats
Dizziness/fainting
Joint and/or muscle pain

Diagnosis

Diagnosis of aortitis is typically made by a detailed medical history, physical examination, as well as various clinical examinations.

Diagnostic imaging, such as computed tomography (CT) angiography, magnetic resonance angiography, or ultrasonography, is often used as part of the diagnosis of aortitis.

Blood tests are typically also ordered to ascertain if there is any inflammation in the body (elevated C-reactive protein, elevated erythrocyte sedimentation rate).

Tissue biopsy may also be performed, which involves the surgical removal of tissue from an affected vessel, which is analysed for signs of inflammation.

Treatment

Treatment differs depending upon the cause of the inflammation. For infectious aortitis, antibiotics are typically prescribed. In instances of aortitis associated with systemic vasculitis, treatment is aimed at suppressing the inflammation through medications such as:

● Corticosteroids (such as prednisone)
● Immunosuppressant drugs (such as cyclophosphamide, azathioprine, mofetil or methotrexate).
● Biological agents (such as rituximab)

Prognosis

Aortitis is a complex and serious medical condition. The prognosis for patients with this condition depends on the underlying cause of the aortitis. Prompt diagnosis and treatment may substantially improve the outcome. Patients with aortitis may experience relapse if associated with an autoimmune condition and will require ongoing monitoring and possibly medication to manage their disease.

Behcet’s Disease

Behcet’s disease is a form of vasculitis that involves inflammation of blood vessels of any size and can affect any organ. Commonly Behcet’s may cause painful mouth and genital ulcers, skin lesions, joint pian and eye inflammation.

Who Gets Behcet’s?

Behcet’s is rare in Australia and New Zealand. It more frequently affects people from the Mediterranian, the Middle East and Asia. The cause is unknown but people with certain immune genes (HLA-B51) may have a greater risk factor of developing the disease. It affects both males and females equally, although the disease severity is typically worse in males. Most patients develop the disease during their 20-30s.

Symptoms

Symptoms and severity differ greatly from person to person. The most common symptoms of Behcet’s include:

● Painful mouth sores
● Sores of the tongue, gums and lining of the mouth
● Painful sore on the genitals
● Eye redness and/or blurred vision
● Joint swelling

Diagnosis

Diagnosis of Behcet’s disease is typically made by a detailed medical history, physical examination, as well as various clinical examinations.

Diagnostic criteria of Behcet’s disease includes 3 episodes of mouth sores within 1 year and 2 of the following:
● Recurring genital ulcers
● Eye inflammation
● Skin lesions that resemble bumps under the skin
● Skin bumps or blisters triggered by a slight injury

Blood tests are typically also ordered to ascertain if there is any inflammation in the body (elevated C-reactive protein, elevated erythrocyte sedimentation rate).

Treatment

Treatment differs depending upon the disease severity, as well as the organ affected. For example

● Corticosteroids (such as prednisone) either oral or topical.
● Immunosuppressant drugs (such as cyclophosphamide, azathioprine, mofetil or methotrexate) for multi-organ or severe disease.
● Biological agents (such as infliximab, etanercept, and interferon alpha)

Prognosis

There is no cure for Behcet’s disease currently, however prompt diagnosis and treatment may relieve many of the symptoms and prevent potentially serious complications such as blindness. Patients with Behcet’s disease may experience relapse and will require ongoing monitoring and possibly medication to manage their disease.

Central Nervous System Vasculitis

Central Nervous System Vasculitis is a form of vasculitis that involves inflammation of the blood vessels that supply the brain and spinal cord, resulting in potentially serious conditions such as loss of brain function, or stroke.

CNS vasculitis is categorised as primary or secondary. Primary CNS vasculitis is inflammation of the blood vessels of the brain in the absence of another cause.

Secondary CNS vasculitis usually occurs along with another autoimmune disease such as Systemic Lupus Erythematosus (SLE), Takayasu Arteritis, Granulomatosis with Polyangiitis (GPA) or even Rheumatoid Arthritis.

Who Gets CNS Vasculitis?

CNS vasculitis is considered rare. The disease onset is typically the fourth or fifth decade and most often occurs in males.

Symptoms

Symptoms vary however common symptoms include:

● Persistent headaches
● Personality changes
● Confusion
● Mini-strokes
● Swelling of the brain (encephalopathy)
● Muscle weakness
● Visual problems
● Seizures or convulsions
● Fever
● Night Sweats
● Dizziness/fainting

Diagnosis

Diagnosis of CNS vasculitis is difficult due to the fact that it shares many signs and symptoms to many other diseases. In addition to a detailed patient history, diagnosis may involve:

● Diagnostic imaging: Such as computed tomography (CT) or magnetic resonance imaging (MRI), or cerebral angiogram.
● Biopsy: Surgical removal of tissue from an affected blood vessel and thereby analysed for inflammation.
● Lumbar puncture: To examine spinal fluid
● Blood tests: To ascertain if there is any inflammation in the body (elevated C-reactive protein, elevated erythrocyte sedimentation rate).

Treatment

Treatment typically involves initially:

● Corticosteroids (such as prednisone)
● Immunosuppressant drugs (such as cyclophosphamide, azathioprine, mofetil or methotrexate).
● Physical, occupational or speech pathology if the brain is affected.

Prognosis

There is no cure for CNS vasculitis at this time, however the condition is treatable. Prompt diagnosis and treatment are essential to prevent potentially life -threatening conditions. Patients with CNS vasculitis may experience relapse and therefore require ongoing monitoring and possibly medication to manage their disease.

Cryoglobulinemia

Cryoglobulinemia is a form of vasculitis that involves the presence of abnormal antibodies (called cryoglobulins) that precipitate out of the blood at cold temperatures. These antibodies clump within blood vessels restricting blood flow and causing damage to muscle, skin, nerves, and organs, particularly the kidneys.

The condition is typically associated with various pre-existing conditions such as Hepatitis C, rheumatoid arthritis, or Sjogren’s syndrome.

Who Gets Cryoglobulinemia?

Cryoglobulinemia often presents in patients that have a pre-existing disease including Hepatitis C, Sjogren’s disease, or even certain cancers.

Cryoglobulinemia is considered a rare disease and may be identified in patients without symptoms. Prevalence is estimated at approximately 1 per 100,000 worldwide. The disease most frequently affects adults over the age of 40 and typically affects females more than males.

Symptoms

Symptoms vary however common symptoms include:

● Skin rash with red or purple discoloration
● Fatigue
● Fever
● Joint pain
● Peripheral numbness or burning sensation
● Muscle pain and/or weakness
● Kidney damage

Diagnosis

Diagnosis of cryoglobulinemia is made by a detailed medical history, physical examination, as well as various clinical examinations including:

● Diagnostic imaging: Such as computed tomography (CT) or magnetic resonance imaging (MRI).
● Biopsy: Surgical removal of tissue from an affected blood vessel or organ biopsy and thereby analysed for inflammation.
● Urinalysis: To look for blood and protein in the urine.
● Blood tests: To confirm the presence of the antibody (cryoglobulin) that clumps at reduced temperatures and assess if there is any inflammation in the body (elevated C-reactive protein, elevated erythrocyte sedimentation rate).

Treatment

Treatment typically begins with treating the underlying condition. For example Hepatitis C often requires suitable anti-virals to inhibit viral replication. Additional treatments may require:

● Corticosteroids (such as prednisone)
● Immunosuppressant drugs (such as cyclophosphamide, azathioprine, mofetil or methotrexate)
● Biological agents (such as Rituximab)
● Plasma exchange to remove the cryoglobulins from the blood.

Prognosis

The prognosis for patients with cryoglobulinemia depends on the underlying disease, which organs are affected, and the disease severity. For some people, no treatment is required as they do not present with symptoms. For others, ongoing monitoring and treatment is required, particularly those with kidney involvement and/or undergoing treatment for the underlying condition.

Eosinophilic Granulomatosis with Polyangiitis (EGPA)

EGPA (Formerly known as Churg-Strauss disease) is a small and medium vessel necrotising disease characterised by extravascular necrotising granulomas (usually rich in eosinophils). The disease can affect any organ, predominantly the lungs, skin, sinuses, cardiovascular system, kidneys, peripheral nervous system, CNS, joints, and GI tract, with occasional lung hemorrhage.

Who Gets EGPA?

The cause of EGPA is unknown. However, an allergic mechanism, with tissue directly injured by eosinophils and neutrophil degranulation products, may be involved. Activation of T lymphocytes seems to help maintain eosinophilic inflammation. The syndrome occurs in patients who have adult-onset asthma, allergic rhinitis, nasal polyposis, or a combination. Antineutrophil cytoplasmic autoantibodies (ANCA) are present in about 40% of cases.

EGPA affects men and women equally, with an incidence of approximately 11-13 cases per million. The average of onset is 40 years of age.

Symptoms

Symptoms range from mild to life-threatening including:

● Fatigue
● Muscle aches and pain
● Weight loss
● Skin rashes
● Chest pain
● Abdominal pain
● Shortness of breath
● Asthma or sinus polyps
● Kidney disease

Diagnosis

Diagnosis of EGPA is made by a detailed medical history, physical examination, as well as various clinical examinations including:

● Diagnostic imaging: Such as computed tomography (CT) or magnetic resonance imaging (MRI) of lungs and/or sinuses.
● Biopsy: Surgical removal of tissue from an affected blood vessel or biopsy of an organ which is then analysed for inflammation.
● Urinalysis: To look for blood or excess protein in the urine.
● Blood tests: ANCA antibody test; Inflammation markers (elevated C-reactive protein, elevated erythrocyte sedimentation rate).
● Heart echocardiogram: All patients with EGPA should be screened for heart involvement.

Treatment

Treatment involves reducing inflammation. Medications include:

● Corticosteroids (such as prednisone)
● Nasal or inhaled steroids
● Immunosuppressant drugs (such as cyclophosphamide, azathioprine, methotrexate)
● Biological agents (such as Mepolizumab or Rituximab)

Prognosis

There is currently no cure for EGPA, however the condition is treatable and well managed, particularly if diagnosed and treated early. Relapse is common and therefore ongoing medical monitoring and treatment may be required.

Anti-GBM Disease

Anti-GBM is a form of vasculitis that affects the blood vessels of the lungs and kidneys. The body mistakenly creates antibodies to the Glomerular Basement Membrane (anti-GBM) resulting in inflammation and bleeding within the lungs and kidneys.

Who Gets Anti-GBM Disease?

Anti-GBM disease is considered rare. The estimated incidence is approximately 1 per 1 million worldwide. The disease typically affects Caucasions and of 2 age groups- young people in their 20s to 30s, and older people in their 60s and 70s. Men are affected slightly more than women and cigarette smokers are at a greater risk of developing the disease.

Symptoms

Symptoms often occur rapidly with early onset of:

● Fatigue
● Muscle aches and pain
● Nausea
● Shortness of Breath
● Lack of appetite
● Fever

Followed by:

● Persistent dry cough
● Coughing up blood (hemoptysis)
● Blood in the urine (hematuria)
● Difficult or painful urination
● Chest pain

Diagnosis

Diagnosis of Anti-GBM disease is made by a detailed medical history, physical examination, as well as various clinical examinations including:

● Diagnostic imaging: Such as computed tomography (CT) or magnetic resonance imaging (MRI).
● Biopsy: Surgical removal of tissue from the lungs or kidney biopsy followed by analysis under the microscope looking for typical tissue damage.
● Urinalysis: To look for blood and protein in the urine.
● Blood tests: Anti-GBM antibody test; Inflammation markers (elevated C-reactive protein, elevated erythrocyte sedimentation rate).

Treatment

Treatment involves reducing inflammation, as well as the aim of removing the anti-GBM antibodies. Medications include:

● Corticosteroids (such as prednisone)
● Immunosuppressant drugs (such as cyclophosphamide)
● Biological agents (such as Rituximab)
● Plasma exchange to remove the harmful anti-GBM antibodies from the blood.
● Kidney dialysis or transplant may be required in those with advanced kidney damage or kidney failure.

Prognosis

The prognosis for patients with Anti-GBM disease requires early diagnosis and aggressive treatment to avoid potentially life-threatening complications. Relapse is uncommon and typically does not require long-term treatment, unless severe kidney damage. It is recommended that cigarette smoke exposure be minimised.

Giant Cell Arteritis

Giant Cell Arteritis involves inflammation of large and medium sized blood vessels such as vessels of the temple area, in the neck, and thoracic aorta. Giant cell arteritis is linked to polymyalgia rheumatica, which causes pain and stiffness in muscles of the neck, shoulders, hips and thighs. A classic symptom of this condition is pain in the tongue or jaw when eating (jaw claudication)

Who Gets Giant Cell Arteritis?

Giant cell arteritis is the most common form of vasculitis in older adults, predominantly affecting people over the age of 50. Women are more likely than men to develop the disease. The incidence is approximately 250-300 per 100,000 people.

Symptoms

The most common symptoms of Giant Cell Arteritis include:

● Fatigue
● Fever
● Unexplained weight loss
● Headache with temple pain or tenderness
● Jaw pain (particularly when chewing)
● Muscle stiffness and joint pain
● Vision loss
● Dizziness
● Left untreated, serious complications may occur including stroke, blindness or blood vessel aneurysm.

Diagnosis

Diagnosis of Giant Cell Arteritis is made by a detailed medical history, physical examination, as well as various clinical examinations including:

● Diagnostic imaging: Such as computed tomography (CT), magnetic resonance imaging (MRI) or positron electron tomography (PET) scans.
● Biopsy: Surgical removal of tissue from a smaller affected blood vessel and thereby analysed for inflammation such as the presence of giant cells.
● Blood tests: Inflammation markers (elevated C-reactive protein, elevated erythrocyte sedimentation rate).

Treatment

Treatment involves reducing inflammation. Medications include:

● Corticosteroids (such as prednisone)
● Immunosuppressant drugs (such as cyclophosphamide, or methotrexate)
● Biological agents (such as Tocilizumab)

Prognosis

The prognosis for patients with Giant Cell Arteritis, particularly if diagnosed and treated early, is good.

Granulomatosis with Polyangiitis (GPA)

GPA (Formerly known as Wegener’s Granulomatosis) is a disease that involves inflammation of the small- and medium-sized vessels, and is considered a systemic ANCA associated vasculitis (AAV). It typically affects the upper and lower respiratory tract, the ears, nose, sinuses as well as the kidneys.

Patients may present with upper and lower respiratory tract symptoms (such as recurrent nasal discharge or epistaxis, cough), followed by hypertension and edema, or with symptoms reflecting multiorgan involvement. Other systemic illnesses are often associated with the inflammatory aspect of the disease including night sweats with or without fever, weight loss, lethargy, joint swelling and pain and malaise.

It should be noted that whilst GPA is considered a systemic disease, the organs involved vary from patient to patient. Some have ‘limited’ disease activity where only the upper respiratory tract is implicated. In contrast, other patients develop kidney inflammation, termed glomerulonephritis.

Who Gets GPA?

The etiology of GPA is not known, however there has been some studies that indicate a correlation to prior exposure to silicon, and/or various microbes including Staphylococcus aureus.

GPA affects approximately 3 per 100,000 people and typically affects people between the ages of 40 and 65. The disease affects males and females equally, and typically affects mostly Caucasions.

Symptoms

The disease of GPA may present in various ways, Initial symptoms include:

● Fatigue
● Fever
● Night sweats
● Unexplained weight loss
● Untreatable sinus infection
● Nose bleeds
● Deafness
● Sinus pain
● Blood in urine
The disease may progress with:
● Lungs: Shortness of breath and/or coughing up blood.
● Skin: Rashes, ulcers and tissue necrosis.
● Eyes: Painful and inflamed eyes.
● Nerves: Loss of sensation, peripheral pain and numbness particularly in the hands and feet.
● Kidneys: Kidney Failure.

Diagnosis

Diagnosis of GPA is made by a detailed medical history, physical examination, as well as various clinical examinations including:

● Diagnostic imaging: Such as chest x-rays looking for lung nodules; computed tomography (CT) or magnetic resonance imaging (MRI).
● Biopsy: Surgical removal of tissue from an affected organ, or renal biopsy which is then analysed for inflammation.
● Blood tests: ANCA antibody test (often positive in GPA patients, although a positive test alone does not confirm diagnosis); Inflammation markers (elevated C-reactive protein, elevated erythrocyte sedimentation rate).
● Urine Tests: To detect red blood cells and/or excess protein to assess for damage to the kidneys.

Treatment

Treatment depends on the organs affected as well as the disease severity. For mild disease conditions, the disease is typically treated with steroids such as prednisone in combination with an immunosuppressant drug such as methotrexate.

The treatment for patients with more aggressive or severe disease may include:

● Corticosteroids (such as prednisone)
● Immunosuppressant drugs (such as cyclophosphamide, or methotrexate)
● Biological agents (such as Rituximab)
● Plasmapheresis
Ongoing evidence is accumulating supporting a long term maintenance regime involving drugs such as methotrexate, azathioprine or Rituximab, to reduce relapse occurrences.

Prognosis

There is currently no cure for GPA, however with early diagnosis and treatment, the disease is often able to be brought into remission. Relapse is common and therefore requires ongoing medical monitoring and treatment.

IgA Vasculitis (Henoch-Schonlein Purpura)

IgA vasculitis is the most common form of vasculitis in children. It can also affect adults. It affects predominantly small blood vessels and capillaries in the skin, joints, bowel and kidneys. If it affects the kidneys it is usually called Henoch-Schönlein Nephritis or Vasculitic IgA Nephropathy. Skin involvement is often indicated by the display of a widespread palpable (small bumps) purpuric (red/purple) rash.

Who Gets IgA vasculitis?

Approximately 90% of patients with IgA vasculitis are children aged between 3 to 10 years. It affects boys more often than girls. The incidence is approximately 10-20 per 100,000 people.

Symptoms

The disease of IgA vasculitis typically presents with muscle aches, fever and headache as well as:

● Nausea, abdominal pain, bloody stools
● Raised purpuric rash predominantly on the buttocks, legs, feet, arms, and abdomen.
● Arthritis of the knees and ankles.
● Blood and/or frothy urine.

Diagnosis

Diagnosis of IgA vasculitis is made by a detailed medical history, physical examination, as well as various clinical examinations including:

● Diagnostic imaging: Such as computed tomography (CT) or magnetic resonance imaging (MRI) of the chest or abdomen.
● Biopsy: Surgical removal of tissue (usually skin) and thereby analysed for inflammation. Skin biopsies may indicate the presence of IgA deposits in small blood vessels. Renal biopsy is also sometimes performed.
● Blood tests: Inflammation markers (elevated C-reactive protein, elevated erythrocyte sedimentation rate). IgA levels.
● Urine Tests: To detect red blood cells and/or excess protein to ascertain a status on the kidneys.

Treatment

Treatment is often for a short period due to the limited nature of IgA vasculitis. Medications may include:

● Antibiotics
● Pain medications
● Anti-inflammatories
● Corticosteroids (usually only for severe disease due to the potential side effects)
● Immunosuppressants (particularly in instances of kidney damage)

Prognosis

There is currently no cure for IgA vasculitis, however the disease typically resolves within a few months. Relapse is common and therefore requires ongoing medical monitoring and treatment.

Kawasaki Disease

Kawasaki disease is the second most common form of vasculitis in children. The disease affects the lymph nodes, mucous membranes, as well as the coronary arteries of the heart.

Who Gets Kawasaki Disease?

Kawasaki disease may affect any age group, however it predominantly affects children under the age of 5 years.

The disease is considered rare, however it affects children of Asian or Pacific Island at a greater rate.

Symptoms

The symptoms of Kawasaki disease presents with a wide range of symptoms including:

● An elevated fever for five days or more
● Skin rash particularly on the trunk and groin areas
● Swollen mouth with cracked lips.
● Blood shot eyes
● Strawberry tongue
● Swollen hands and feets
● Joint pain.
● Skin peeling.
● Stomach pain.
● Heart complications.

Diagnosis

Diagnosis of Kawasaki disease is made by a detailed medical history, physical examination, as well as various clinical examinations including:

● Diagnostic imaging: Such as Chest X-rays
● Biopsy: Surgical removal of tissue from an affected blood vessel or organ and thereby analysed for inflammation.
● Blood tests: Inflammation markers (elevated C-reactive protein, elevated erythrocyte sedimentation rate). Infectious diagnostics to rule out other diseases with similar symptoms such as scarlet fever or measles.
● ECG: To monitor heart damage.

Treatment

Prompt treatment is required to minimise severe complications, particularly related to the heart. Medications may include:

● High dose Immunoglobulin
● Antipyretics
● Anti-inflammatories
● Pain medications
● Anti-inflammatories
● Corticosteroids (usually only for severe disease due to the potential side effects)
● Immunosuppressants (particularly in instances of severe disease)

Prognosis

The prognosis for people with Kawasaki disease is good. Most people fully recover. A proportion of people develop serious heart complications. As such, early diagnosis and treatment is essential. Follow up check ups with medical specialists are often required, particularly those that have heart complications.

Microscopic Polyangiitis (MPA)

MPA is is a small vessel form of vasculitis associated with ANCA (AAV). MPA predominately affects the kidneys, lungs, nerves, skin, and joints. It may begin as a pulmonary-renal syndrome with rapidly progressing glomerulonephritis and alveolar hemorrhage, but the pattern of disease depends on the organs affected.

Who Gets MPA?

MPA can affect people of any age, however it most commonly affects people in their 50s onwards. It affects both men and women, though affects men more frequently than women.

The incidence of MPA is considered rare with an estimated incidence of 1-3 per 100,000 people.

Symptoms

The symptoms of MPA include general system symptoms such as fever, weight loss, myalgia, and arthralgia.The disease may also present with:

● Kidney inflammation including blood in urine
● Skin rashes
● Shortness of breath
● Persistent cough
● Nerve involvement (Including loss of sensation, pain and weakness)
● Joint and muscle pain
● Abdominal pain
● Eye irritations.

Diagnosis

Diagnosis of MPA is made by a detailed medical history, physical examination, as well as various clinical examinations including:

● Diagnostic imaging: Such as chest X-rays, CT scans
● Biopsy: Surgical removal of tissue from an affected blood vessel or organ and thereby analysed for inflammation.
● Blood tests: Inflammation markers (elevated C-reactive protein, elevated erythrocyte sedimentation rate).
● Urine Analysis: To detect the presence of red blood cells, which may indicate kidney inflammation.

Treatment

Prompt treatment is required to minimise severe complications, to the lungs and kidneys. Medications include:

● Corticosteroids (Such as prednisone)
● Immunosuppressants (Such as cyclophosphamide)
● Biologicals: (Such as Rituximab)
● Plasmapheresis (For those with kidney involvement)
Ongoing evidence is accumulating supporting a long term maintenance regime involving drugs such as methotrexate, azathioprine or Rituximab, to reduce relapse occurrences.

Prognosis

There is currently no cure for MPA, however with early diagnosis and treatment, the disease is often able to be brought into remission. Relapse is common and therefore requires ongoing medical monitoring and treatment.

Polyarteritis Nodosa (PAN)

PAN is a rare form of vasculitis that affects medium sized blood vessels, such as the bowel, skin and kidneys.

Who Gets PAN?

PAN is a rare disease with an estimated incidence of 3-5 per 100,000 people. The disease is more common in men than women, and affects people predominantly between the ages of 45 and 65 years.

Symptoms

The symptoms of PAN is wide and varied. It often precipitates with night sweats, fever, weight loss, skin sore and muscle and joint pain and may then result in:

● Testicular pain
● Skin nodules
● Abdominal pain
● Chest pain
● Numbness in the hands and/or feet.
● Elevated blood pressure

Diagnosis

Diagnosis of PAN is made by a detailed medical history, physical examination, as well as various clinical examinations including:

● Diastolic blood pressure greater than 90 mm/Hg
● Elevated blood urea nitrogen (BUN) or creatinine level unrelated to dehydration or obstruction
● Presence of hepatitis B surface antigen or antibody in serum
● Arteriogram demonstrating aneurysms or occlusions of the visceral arteries
● Biopsy of small- or medium-sized artery containing polymorphonuclear neutrophils

Treatment

The aim of treatment is to primarily reduce inflammation. Medications include:

● Corticosteroids (Such as prednisone)
● Immunosuppressants (Such as cyclophosphamide)
● Anti-TNF therapies
● Medications for the treatment of elevated blood pressure

Prognosis

There is currently no cure for PAN, however with early diagnosis and treatment, the disease is often able to be brought into remission. Relapse is common and therefore requires ongoing medical monitoring and treatment.

Takayasu Arteritis

Takayasu Arteritis in a type of large vessel vasculitis, such as the aorta and its main branches.

Who Gets Takayasu Arteritis?

Takayasu Arteritis affects approximately 1 in 200,000 people, and affects predominantly females between the ages of 15 to 40 years of Asian ethnicity.

Symptoms

The symptoms of Takayasu Arteritis include general systemic symptoms such as fever, weight loss, myalgia, and arthralgia.The disease may also present with:

● Visual disturbances
● Chest pain
● Headaches
● Dizziness
● Shortness of breath
● Muscle weakness
● Stroke

Diagnosis

Diagnosis of Takayasu Arteritis is made by a detailed medical history, physical examination, as well as various clinical examinations including:

● Diagnostic imaging: Such as CT angiography, MRI angiography.
● Physical Exam: Such as blood pressure, heart sounds, pulse.
● Blood tests: Inflammation markers (elevated C-reactive protein, elevated erythrocyte sedimentation rate).

Treatment

Prompt treatment is required to minimise severe complications,

● Corticosteroids (Such as prednisone)
● Immunosuppressants (Such as cyclophosphamide)
● Biologicals: (Such as Infliximab, and Tocilicumab)
● Surgery (May be required in cases where arteries have become blocked, narrowed or formed aneurysms).

Prognosis

There is currently no cure for Takayasu Arteritis, however with early diagnosis and treatment, the disease is often able to be brought into remission. Ongoing medical monitoring may be required.

Urticarial Vasculitis

Urticarial vasculitis is a form of vasculitis that predominantly affects small blood vessels. There are two forms of urticarial vasculitis:

● Hypocomplementemic: Low levels of complement proteins (particularly C1q) resulting in skin hives, as well as systemic involvement such as the joints, kidneys, lungs, eyes and gastrointestinal tract.
● Normcomplementemic: Normal levels of complement proteins with little systemic involvement, though still typically presents with skin hives that can itch, and leave skin discoloration.

Who Gets GPA?

Urticarial vasculitis typically affects adults between the ages of 30 to 40 years. Women are twice as likely to be affected than men. The incidence and/or prevalence rate of urticarial disease is not well documented, however is considered rare.

Symptoms

The disease of urticarial vasculitis typically begins with skin lesions and hives (urticaria). The coloration may be red/purple indicating bleeding under the skin. In systemic disease, more common symptoms include:

● Fatigue
● Fever
● Night sweats
● Abdominal pain
● Shortness of breath (dyspnea)
● Inflamed eyes
● Cardiac involvement
● Kidney involvement

Diagnosis

Diagnosis of urticarial vasculitis is made by a detailed medical history, physical examination, as well as various clinical examinations including:

● Diagnostic imaging: Such as chest x-rays looking for lung nodules; computed tomography (CT) or magnetic resonance imaging (MRI).
● Biopsy: Surgical removal of tissue from an affected blood vessel or organ and thereby analysed for inflammation.
● Blood tests: Complement levels; Inflammation markers (elevated C-reactive protein, elevated erythrocyte sedimentation rate).
● Urine Tests: To detect red blood cells and/or excess protein to assess for damage to the kidneys.

Treatment

Treatment depends on the disease severity and the organs affected. With skin hives, treatment may include anti-histamines, non-steroidal anti-inflammatories, or analgesics.

For systemic disease, treatment may include:

● Corticosteroids (such as prednisone)
● Hydroxychloroquine
● Colchicine
● Dapsone
● Immunosuppressant drugs (such as cyclophosphamide, or methotrexate)
● Biological agents (such as Rituximab)

Prognosis

There is currently no cure for urticarial vasculitis. In instances of chronic urticarial vasculitis or systemic involvement, ongoing medical monitoring and treatment are often required.