From the National Magazine

Self Help in Vasculitis

Prof Kuntal K Chakravarty, Consultant in Rheumatology,
Harold Wood Hospital, Romford, Essex

The following has been extracted with the kind permission of Prof. Chakravarty, from a booklet written to try to explain what Vasculitis is in easy to understand terms. It also covers the types of investigations a newly diagnosed patient can expect and the types of treatment used to bring about remission. It covers many of the questions that people want to ask but don't like to bother their doctor with, along with tips to help manage drug therapy and how to keep as healthy as possible.

What is meant by Vasculitis?
When you have "itis" at the end of a word it means inflammation. Therefore Vasculitis is an inflammation of the blood vessels. Blood vessels are the Arteries which carry the pure oxygenated blood to the organs and the body's extremities, and the Veins which drain the impure blood from the organs and extremities. In some circumstances, smaller vessels called Capillaries, can also be affected.

Every organ in the human body needs a good blood supply to function properly. With Vasculitis, because the blood vessels are inflamed reducing the amount of blood getting through, this affects the function of the organ which they supply. This is why any organ in the human body can be affected because every one of them has a blood supply. Hence Vasculitis is known as a "multi-system disease".

The severity of Vasculitis differs from patient to patient and the damage it causes depends on the organs involved and the severity of the Vasculitis. Sometimes this illness can predominantly affect the nose or the windpipe, causing significant problems with recurrent bleeding, crusting or blocking of the nose. In some cases it can affect the lungs causing breathing problems, or the kidneys. The doctor treating your Vasculitis will be well aware of these symptoms and will know that there are many other reasons for breathlessness so no one should worry that all shortness of breath symptoms are necessarily due to Vasculitis. Sometimes Vasculitis can predominantly affect the skin and bowel as is seen in cases of a particular type called H.S. Purpura.

There are several sub types of Vasculitis and it is the responsibility of your doctor and the specialist to find out which one has been causing problems in your case. Please do not worry, just try and co-operate with your doctors to make a quick diagnosis, which will help in instigating the right treatment quickly.

What are the types of Vasculitis?
Vasculitis can generally be divided into either Primary or Secondary. When Vasculitis occurs in a person already suffering from Rheumatoid Arthritis or Lupus, it is called Secondary Vasculitis. When it occurs without any pre-existing diseases it is called Primary Vasculitis. There are several types of Primary Vasculitis such as Wegener's Granulomatosis, Polyarteritis etc.

Sometimes vasculitis is also classified by the size of the blood vessel involved. When it affects a big blood vessel, it is called Large Vessel Vasculitis and when it affects a small blood vessel it is called Small Vessel Vasculitis. The classical examples of such vasculitis are Temporal Arteritis (Large Vessel Disease) and Wegener's Granulomatosis (Small Vessel Disease).

How common is Vasculitis?
The illness arising from Vasculitis is fortunately very rare and the prevalence rate varies from one geographical area to another. The rate at which new cases are diagnosed is also very variable and we are recognising this disease more now, due to several reasons. One of the major reasons is the greater awareness among doctors, and the availability of new diagnostic tests.

What type of person gets Vasculitis?
There is no specific type of person who would necessarily develop this disease. However, there are some certain underlying diseases that may predispose people to develop Vasculitis. For instance, people who already suffer from Rheumatoid Arthritis, Lupus or another similar type of disease.

Anyone can be unfortunate enough to get Vasculitis and in some cases a predisposing cause can be found, for example a preceding infection or drug therapy such as recent treatment with some antibiotics. There are some cases reported occurring after a viral or non viral infection. It is not clear who are more likely to get this disease in the absence of identifiable causes, but some research is ongoing into this.

Vasculitis is neither infectious nor contagious and the disease has no obvious connection with any hereditary factors that are known as yet.

Why are some General Practitioners unaware of this disease?
There are many reasons, but the crucial one is the fact that this disease has always been considered rare until very recently when better screening via blood tests became available. There was also less awareness of this disease but that scene has been changing very rapidly due to the number of GP's who have been informed through lectures and direct involvement locally with a group of doctors called the "Essex Vasculitis Research Association" or EVRA. It is the aim of EVRA and the support groups to make GP awareness a high priority and hopefully more cases will be diagnosed in the early stages.

Common symptoms of Vasculitis
The illness can present either abruptly or very gradually, with the development of symptoms over days or weeks. The commonest "abrupt" presentation, is Skin Rash, which can be itchy or non itchy and can affect upper or lower limbs or even the whole body.

In small Vessel Vasculitis (Wegeners Granulomatosis) most people feel vaguely unwell without any specific symptoms, but after a time may develop symptoms related to the upper airways such as the nose or sinuses. There can be nasal blocking, crusting and spontaneous bleeding but the symptoms can be extremely varied.

Rarely, in certain types of Vasculitis, one can get painful mouth or genital ulceration. Also, rarely, one can get involvement of internal organs such as lungs, kidneys, liver etc. In cases of Large Vessel Vasculitis (Temporal Arteritis) patients may complain of headaches on one side of the head for some time which do not respond to simple Paracetamol treatment. Very occasionally patients can complain of diminishing eye sight, which should be mentioned to the doctor immediately.

In certain patients Vasculitis can be the presentation of a heart attack or stroke. However, the diagnosis of Vasculitis is always entertained by doctors with the background of an appropriate history.

Is tiredness a symptom of this disease?
In Vasculitis, tiredness may be the only symptom that presents itself and if the disease is active, the patient is likely to feel more tired than usual. However, tiredness is also occasionally felt when the disease is not active, usually because people tend to try and regain normal activity rather to quickly, therefore allowing less time for the body to regain strength. It has to be remembered that Vasculitis is a serious illness, particularly if someone has a serious involvement of internal organs such as kidneys, liver or lung.

What tests are needed to diagnose Vasculitis?
There are several tests that doctors might like to do, depending on the clinical symptoms that the patient presents with. Most common test is a biopsy of tissue (ie skin, Lung, Kidney) is required to confirm the diagnosis because without this, the diagnosis can remain doubtful.

It depends on the organ affected as to who does the biopsies, for example, a skin biopsy is normally carried out by a Dermatologist (a specialist in skin diseases) but it can also be done by a physician including a Rheumatologist (specialist in bone and joint diseases).

The biopsied tissue is then sent to a specialist called a Histopathologist, who looks at it under a microscope, after treating it appropriately with the right form of chemicals which highlight the abnormality. It can take a few days to do the special staining so there is often a slight delay in confirming the diagnosis.

Doctors also like to do blood tests, this is for two main reasons; Firstly to diagnose the condition and to exclude other conditions which may mimic Vasculitis and secondly to ascertain which organs are affected and to what degree. A number of blood tests are necessary in the beginning, depending on the patients symptoms and there is often a long term requirement of blood tests to assess how the disease is responding to the treatment.

Quite often, when seeing their doctors, patients may be asked if they mind the doctor taking photographs of skin rashes and other skin abnormalities. This is perfectly normal because this helps when referring back through patient notes and also to help with the study of Vasculitis.

Is Vasculitis curable?
There are types of Vasculitis which one may expect to be cured, but the major types like Wegener's Granulomatosis, Polyarteritis etc. are not necessarily curable. However, they can be effectively treated and with a reasonably good prognosis if diagnosed at the right time. You should try and think of it in the context of other more common diseases such as Diabetes or High Blood Pressure, none of which are curable and the patient has to take medication for life. So try to be optimistic and think along these lines. A positive approach is half the battle.

What is the prognosis?
The prognosis can be very variable. No two patients with the same disease will necessarily have the same outcome. There are some patients that do not need treatment despite a positive diagnosis, partly because they feel so well, but the reverse can also be true.

The actual outcome of the disease depends on a number of factors, some of which are related to the side effects of the drug therapy. Therefore, the treatment and the outcome depends on both the severity of the illness as well as the side effects of the drug therapy. Sometimes it becomes very difficult for doctors to distinguish between these two but in most cases they get the right results.

What is meant by Remission and Relapse?
After the initial acute phase of Vasculitis there are two other phases called Relapse and Remission phase. Once the disease has been initially treated and brought 'Under Control' (when the inflammation in the blood vessels quietens down), this is called Remission and with the right drug therapy it is hoped that most cases will reach this stage and be maintained. Sometimes the patient can develop new symptoms of the disease or other complications and this is known as Relapse. As long as you keep all your hospital appointments and tests, any relapse should be caught quickly and hopefully reversed.

What drugs are commonly used to treat this condition?
There are quite a few drugs used to treat Vasculitis but the common ones are:

Cyclophosphamide: A very strong drug given either via a vein or in tablet form to help suppress the immune system.

Steroids: Quite often Prednisolone, initially used to reduce inflammation and often used in small doses to help maintain remission.

Methotrexate: This can suppress the immune system and also act as anti-inflammatory, often used to help maintain remission.

Chlorambucil: This can suppress the immune system and also act as anti-inflammatory, often used to help maintain remission.

Will I have to take drugs for the rest of my life?
With Vasculitis you should be prepared to take drugs for the rest of your life. When the disease is active you will have to take drugs regularly. However, there may be times when the disease is in remission and the doctor is happy for you not to take daily medication. Perhaps it is useful to again think of diabetes, which is a far more common disease than Vasculitis and realise that the treatment for that is for life. So think positive and as long as the disease is not causing any damage and the drug is working to the desirable extent, one should not worry too much.

Does diet play a role in the treatment of Vasculitis?
There may be some role of the diet in the treatment of Vasculitis, but as yet, it has not been researched. It may be possible that research into this will be done in the future, but there is a basic problem. Because the disease is fairly acute, no research at present can produce as quick a result as desired, therefore doctors have no knowledge or experience of any particular diet that is helpful.

Having said this, as with any illness, a healthy balanced diet is essential. Fresh fruit and vegetables and less fat in the diet are good, as is making sure that you take in plenty of calcium if you are on steroids, as these drugs can cause brittle bones.

If you are having cyclophosphamide treatment, a diet rich in iron is also helpful to prevent anaemia.

Diet is basically common sense, a healthy diet will benefit everyone, with or without illness.

Will I ever regain Normal Health?
The ability to regain normal health depends on the severity of the disease and also the rate at which you respond to the treatment. If the disease is diagnosed fairly early and treatment is administered at an early stage, you can normally expect to see some improvement. If that improvement is maintained, then the outlook is likely to be good and the chances of getting back to a normal life very possible. However, sometimes the response to treatment may not be as good as expected and the outcome may lead to some other problem which interferes with getting back to a normal life.

You must be prepared to make some life changes, some will be minor, others not so. You should also remember that one persons idea of a normal life, may not necessarily be normal to another.

Smoking & Drinking
People with Vasculitis should never smoke. The whole point of treating you, is to prevent anymore damage to your blood vessels and smoking can actually constrict them further.

Drinking should be discussed with your doctor. People taking steroids should try and avoid alcohol as the drugs can cause some stomach problems such as indigestion which alcohol can make worse. If your doctor is happy that your drug therapy or general health is not going to be compromised by alcohol, then it may be taken in moderation.

Pregnancy
It is very unlikely for someone to fall pregnant while the disease is active or if they are receiving treatment with certain drugs such as Methotrexate or Cyclophosphamide. These drugs do cause some reduction in terms of fertility and your doctor will discuss this with you before starting treatment.

The effects of the drugs and the disease on a successful pregnancy is not clearly known from the scientific data available. It is generally advised to avoid pregnancy when the disease is active or when receiving treatment. You will be advised by your doctor, to use contraception while receiving treatment with Methotrexate or Cyclophosphamide and you should discuss any worries about this issue with your doctor.

Once remission has been reached, if a pregnancy is still desirable, then this should be discussed with your doctor and planned accordingly. This is best done if treatment is stopped altogether.

How to help yourself or others with Vasculitis
There is no better way to help yourself in this illness than listening to your doctors advice and following it very carefully. A good relationship with your doctor is essential and you should not be afraid to speak to him about any problems or concerns that you have.

A good healthy diet, plenty of rest and gentle exercise to avoid stiffening joints is a good habit to adopt. Listen to your body and rest when it tells you to. Don't try to fit too many things into your day but pace yourself and you will feel better for it.

Making sure that you take your drugs regularly and at the right times is very important. A good way to remember what you have to take, is to buy a calendar with daily boxes on it and different coloured sticky dots, available from any stationers. You can then use one colour for each drug and place the dots on the calendar, so you can see at a glance what medication you have to take that day. Another way (instead of, or to compliment the calendar) is to buy a drug wallet with seven little boxes in it so that you can prepare your weeks drugs in advance.

As for helping others, the best way is by sharing your experience of your illness, treatment and anything you found helpful yourself. This can effectively be done by joining a patient support group, such as The William Godfrey Vasculitis Patient Support Group, which is located in Romford. The contact details and other information can be found at the end of this article.

How can patients help their doctors?
This can be done in very practical ways. Firstly it is good to know a little bit about the illness and it's effects. This can be done by reading the literature provided by the support groups and the doctors at the hospital. Secondly you should keep a good record of all treatment that you receive, including the date of starting specific treatments such as Methotrexate, Cyclophosphamide and Steroids. Taking this with you to appointments, can help the doctors, so they don't have to search through a huge pile of notes to see when you started taking a particular drug. Lastly documenting, in the form of a diary with dates etc., any symptoms that may be worrying you. It is often the case that you will see your doctor and forget to mention certain things, but with it written down you won't have to worry. It is important to realise that all information you have about your disease, is extremely important in the long term.

Research into Vasculitis
As the incidence of Vasculitis is growing, so more research is being done. There are a number of research projects on going locally, nationally and internationally. If you would like to help in any of this you can always contact your consultant, doctor or support group to find out more about it.

Treatment of Vasculitis
In most cases, the treatment is given after the diagnosis is confirmed by a tissue biopsy except in rare events when a biopsy cannot be possibly be done due to associated risks.

The three reasons why treatment is given are as follows:

In the early stages of the disease the drugs that are commonly used are Cyclophosphamide and Steroids. However, if the disease is mild or there is a problem in giving drugs like Cyclophosphamide, then other options such as Methotrexate are explored. Even though you may have read about these drugs and you have your own opinion as to which one you would prefer, it is best to leave it in the hands of your doctor, as long as the side effects are made reasonably clear to you. It is your responsibility, however, to bring up any specific concerns that you may have over the choice of drugs.

The major risks associated with these 'long tested drugs' are mentioned in the 'Information Leaflet for Individual Drugs' published by the Arthritis Research Campaign, which should be available from the clinics. The leaflet is usually given to the patient to read before the drug is started unless it is a dire emergency, when to take consent may cause a serious waste of time and endanger lives. Normally, the doctor will ask for a formal, informed consent from the patient, so that all concerned will know that you had read the literature and understood the contents.

If your treatment is started on a planned basis and not an emergency, you should be offered the leaflet to read. If this is not offered, then please feel free to ask for one before starting the treatment. It is important to you and your doctor to become and stay informed.

Steroids are the main treatment in Vasculitis and are life saving if used under proper guidance. They are always used in the early phase of treatment either by tablet form or through the vein. It is important to ask your doctor any questions you have about this drug.

Side Effects
As with any drug, there are some side effects with some of these very strong drugs, such as Cyclophosphamide and Methotrexate. The most common side effects are:

With any of these, the severity of the symptoms depends on how high a dose of the drug you are taking. Bladder problems can be lessened by drinking plenty of water throughout the day and, if you feel very sick, drugs can be given to alleviate that.

All the side effects of these drugs can be found in the literature provided by the Arthritis Research Campaign and doctors are always happy to discuss any concerns you may have.

Further reading on Vasculitis "An information booklet on Vasculitis" by the Arthritis Research Campaign available from The William Godfrey Vasculitis Patient Support Group.

Helplines: Dr K Chakravarty (Academic secretary) 01708 785019 - answer phone service available, your call will be returned within a reasonable time.

William Godfrey Vasculitis Patient Support Group website www.angelfire.com/va2/wg
email: wgvpsg@btinternet.com

'New Advancements' since the publication of the original booklet 4 years ago.

New drugs are being developed for the treatment of Vasculitis and some are already tested with success. New drugs are classified as immunotherapies and these could include drugs like anti B cell ie. Rituximab and also conventional anti tumour necrosis factor therapies such as Infliximab, Etanercept and Adalimumab, often used in the treatment of refractory rheumatoid arthritis.

High dose therapy with Cyclophosphamide and steroid still remains one of the initial choices in the treatment of systemic vasculitis. Deoxypergualin is another drug which is going through clinical trial in some cases of vasculitis.

One of the things we have to remember is the fact that all these "smart" drugs have complications too and there use should remain in the hands of experts.

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