What is vasculitis?
There are a number of vasculitis diseases (the vasculitides). All are characterised by inflammation of the blood vessels in one or more organ(s). The vasculitides are diseases involving the immune system whereby the immune system no longer provides defence against infection or other triggers and, therefore, the immune system fights itself. For further information see: What is Vasculitis?
What is inflammation?
The process used by the body to protect against or eliminate foreign bodies, bacteria or viruses. The usual signs of inflammation include redness of the skin, swelling, feeling of warmth or pain in the affected areas.
What are the different types of vasculitis?
The vasculitides can affect small, medium or large arteries. The main diseases are:
Large sized arteries
Middle sized arteries
Small sized arteries
- Eosinophilic Granulomatosis with Polyangiitis (previously known as Churg-Strauss Syndrome)
- Microscopic Polyangiitis
- Granulomatosis with Polyangiitis (previously known as Wegener’s Granulomatosis)
- Henoch-Schönlein Purpura
Less common vasculitides
- Anti GBM (Goodpastures Disease)
- Behçet’s Syndrome
- Buerger’s Disease
- Central Nervous System Vasculitis
- Cogan’s Syndrome
- Cryoglobulinemia and Cryoglobulinaemic Vasculitis
- Urticarial Vasculitis
What are the symptoms of vasculitis?
As there are a number of different vasculitis diseases and as they can attack most organs of the body the symptoms differ from vasculitis to vasculitis and from patient to patient. Some general symptoms include: tiredness, weakness, loss of appetite, weight loss and fever. See: Individual Diseases for symptoms related to specific. For general symptoms related to vasculitis see: Symptoms of vasculitis.
What drugs are used to treat vasculitis?
There are two stages in the treatment of vasculitic disease. The first stage is controlling the disease process and the second is maintaining remission and preventing relapse. Depending on the disease, the patient and the severity of the disease the initial stage drug regime can include low dose chemotherapy (cyclophosphamide), co-corticosteroids (prednisolone), methotrexate or Azathioprine – see Glossary of Drugs. For maintenance of remission and to prevent relapse the drug regime includes methotrexate, azathioprine, cellcept (mycophenolate mofetil) and low dose co-corticosteroids (prednisolone) (see individual disease sections). Other drugs are used where patients are intolerant to the usual drugs or where these drugs have not been successful in controlling the inflammation, e.g. Rituximab.
What causes vasculitis?
In most cases of vasculitis the exact cause is not known. However recent research suggests that in most cases of vasculitis a genetic or environmental trigger (or a combination of both) may be the cause. For further information see: Causes of vasculitis.
How common is vasculitis?
The vasculitides are rare diseases, with 10-15 new cases a year (per million population) being diagnosed with ANCA associated vasculitis. Some types of vasculitis are extremely rare and you should discuss this with your consultant
Who are affected?
Some of the vasculitides affect males and females in equal numbers, whilst others tend to affect a higher proportion of males. The usual onset for vasculitis diseases generally is from age 50 onwards, but young people and infants can be affected.
What is ANCA?
Anti-neutrophil-cytoplasmic antibody. In ANCA associated vasculitis the B-cells produce antibodies that are directed against proteins found on the surface of neutrophils (a type of white blood cell). Read more about ANCA.
Does a negative ANCA mean I don’t have vasculitis?
No, some patients can be ANCA positive and others ANCA negative. Individual patients may be positive at one blood test and negative at another. The physician uses the ANCA test as a guide to treatment, along with other test results and by examination and discussion with the patient.
Should the vasculitis patient avoid taking other drugs
Drugs such as “over the counter” drugs should not be taken unless this has been discussed with the doctor or pharmacist.
Is vasculitis hereditary?
There is a possibility of a genetic link which might predispose individuals to developing one of the vasculitic diseases, but it is not considered that genetic predisposition alone is a factor.
Why is vasculitis so difficult to diagnose?
There are a number of vasculitic diseases and each can involve any number of organs in the body. In addition many of the symptoms the patient presents with are similar to many other illnesses or diseases. Vasculitis can mimic other diseases. As the treatment for vasculitis is by prescribing toxic drugs there are many diagnostic tests to be undertaken to rule out other diseases and to confirm the presence of vasculitis.
Is vasculitis infectious or contagious?
Is there a cure for vasculitis?
Although some of the vasculitides are self-limiting the majority cannot be cured. Research continues into the causes of vasculitis and treatments. Vasculitis is controlled by maintenance therapy.
How often should I see my consultant?
In the initial stages of treatment patients are seen at regular intervals by their consultants. When remission is achieved the period between consultations is increased, but even where patients are drug free they should still be monitored regularly.
Which consultants treat vasculitis?
This depends on the organs involved, but the main specialties include: nephrologist (renal), rheumatologist, ear, nose and throat, paediatrician, ophthalmologist, neurologist and respiratory physicians.
Do consultants specialise in vasculitis?
As the vasculitic diseases are rare most patients are treated by consultants whose main specialties include: nephrologist (renal), rheumatologist, ear, nose and throat, paediatrician, ophthalmologist, neurologist and respiratory physicians. However, a number of these consultants also specialise in treating patients with vasculitic diseases.
Where will I receive my treatment?
Usually patients are seen at local or area hospitals. Some patients are seen at national centres dealing with vasculitis. Where the patient is treated should be discussed between the patient and the GP.
Are there centres which specialise in treating vasculitis?
There are a number of national centres which specialise in treating vasculitis. Referral to these centres should be discussed with the GP.
Is research being undertaken into vasculitis?
Yes. A considerable amount of research has been undertaken nationally and internationally into the causes, treatment and effects of vasculitis. National and international research projects are currently ongoing. You can read about current research into vasculitis at: Research
How often should I have blood tests?
In the initial stages bloods monitoring is undertaken at regular intervals depending on the drug regime prescribed. The periodicity of blood testing when the patient is in remission depends on the patient, the maintenance therapy prescribed and other medical factors. See: Glossary of Blood Test Monitoring
How do I interpret the results of blood tests?
Your physician will interpret the results and discuss these with you, it is also possible to request a copy of your blood test results. There are no absolute levels for the various tests but these can vary between patients and the laboratory doing the analysis. It is the change found from test to test in the individual which is important.
What tests/procedures are undertaken?
These depend on the vasculitis and the symptoms. See Glossary of Procedures.
What is a biopsy?
This is a procedure whereby a small piece of tissue is taken from the affected area or organ, e.g. the skin or the kidney. The tissue is then examined under a microscope. A biopsy is the most conclusive way of ascertaining whether vasculitis is present.
What is a flare?
A flare is where the disease has been relatively stable on maintenance therapy but there is a sudden change in the original symptoms or new symptoms are reported.
What is remission?
After the initial treatment period, when the physician considers the disease is drug/medication controlled he/she may consider the patient to be in clinical remission. If the disease is considered to be in remission or inactive without drugs/medication, this is normally classed as full remission
Can vasculitis be fatal?
In some cases of severe disease if not diagnosed early and not treated correctly. With early diagnosis and appropriate treatment vasculitis is now rarely fatal. Many milder cases may cause damage to organs or discomfort but are not life-threatening.
Is vasculitis linked to cancer?
No. However, there is a link between the use of chemotherapy and the onset of bladder cancer in later life, but modern dosages of chemotherapy have reduced this possibility considerably.
Is vasculitis like AIDS?
No. Vasculitis involves the immune system being over-active. AIDS is an acquired disease where the immune system is under-active. AIDS can also be transferred to others whereas vasculitis cannot.
Can the vasculitis patient have another autoimmune disease?
Should I have vaccinations?
Patients taking immunosuppressants should NOT receive LIVE vaccines. However, flu and pneumonia vaccines are recommended for vasculitis patients. Where the patient is in doubt about a particular vaccine this should be discussed with the GP. For further information about vaccinations see: Vaccinations
Can vasculitis affect fertility?
Taking cyclophosphamide and similar drugs may render the patient less fertile or infertile. See: Fertility and Vasculitis
Is it advisable to use contraception during treatment?
Whilst taking drugs such as cyclophosphamide it is essential to use effective contraception as the drugs will cause damage to the unborn child.
Can vasculitis affect the eyes?
Yes some patients can have problems with their eyes – see: Vasculitis and the Eyes
Can vasculitis affect the hearing?
Yes some patients can have problems with their hearing – see: Vasculitis and the Ears
Can children be diagnosed with vasculitis?
Unfortunately a number of children are diagnosed with vasculitis each year. See: Vasculitis in Children
Is it possible to manage the pain of vasculitis?
There are a number of ways to help manage pain. See: Managing Chronic Pain
Is it possible to manage low mood and depression caused by vasculitis?
Many vasculitis patients suffer from low mood or depression. This is a normal phenomenon and is often transient. See Low mood and depression.
What about oral health for the vasculitis patient?
It is important to ensure good oral hygiene for all vasculitis patients. This is especially the case where there are pre-existing dental problems which may get worse due to steroids or immuno-supressants. See: Oral Health
What about diet?
Some patients may be prescribed a special diet and it is essential to keep to this eating regime. Diet-controlled food intake is important since too much will cause weight gain in addition to that experienced by the use of steroids. For further information see: Diet
What about alcohol?
Alcohol should not be taken with some of the drugs used to treat vasculitis. Where alcohol consumption is permitted this should be in moderation.
Is there an increased chance of becoming diabetic?
Some patients may become diabetic and this will be controlled by drugs and a healthy diet.
Should I exercise?
The vasculitis patient can easily become fatigued, but light regular exercise, walking, swimming etc will ensure your muscles stay strong and flexible. Any exercise should be within your capabilities.
Should I have a DEXA (bone) scan?
Patients taking high doses of steroids are at risk of developing osteoporosis. Therefore, periodic bone scans are recommended. The risk of developing osteoporosis and the best way to treat or monitor this should be discussed with the medical team.