About Vascultis

What is vasculitis | Who is affected | What are the causes | What are the symptoms | Diagnosis | What is the outlook | Ongoing care | Common medications | Emotional issues and support | Tips for patients with vasculitis

What is vasculitis

Vasculitis (plural: vasculitides) refers to a heterogeneous group of disorders that are characterized by inflammatory destruction of blood vessels. Conditions covered by vasculitis include

  • Behçet's syndrome
  • giant cell arteritis
  • polymyalgia rheumatica
  • Takayasu's arteritis
  • cutaneous vasculitis
  • polyarteritis nodosa
  • Kawasaki disease
  • Berger disease
  • Churg–Strauss syndrome
  • cutaneous vasculitis
  • Henoch–Schönlein purpura
  • microscopic polyangiitis
  • Wegener's granulomatosis

Vasculitis can affect people of all ages. The outlook for people who have vasculitis varies depending on both the type and severity of the vasculitis. Treatment is often very effective if vasculitis is diagnosed and treated early. In some cases, it may be a chronic problem, requiring ongoing treatment, or it may come back (“flare”) after a period of remission. In more severe cases, treatment may not help, and vasculitis can lead to disability or even death.

Although the diseases share many of the same symptoms and treatment courses, each is different and patients must learn as much as possible about their individual disease. Additional research is needed to learn more about the various types of vasculitis and their causes, treatments and remission patterns.

According to the size of the vessel affected, vasculitis can be classified into:

  • Large vessel: Behçet's syndrome, giant cell arteritis, polymyalgia rheumatica, Takayasu's arteritis
  • Medium vessel: cutaneous vasculitis, polyarteritis nodosa, Kawasaki disease, Berger disease 
  • Small vessel: Churg–Strauss syndrome, cutaneous vasculitis, Henoch–Schönlein purpura, microscopic polyangiitis, Wegener's granulomatosis

Vasculitis_Image_1
Image source: Vasculitis-Patient.com

Who is affected by vasculitis?

Vasculitis can affect people of all ages from childhood to adulthood. There are some types of vasculitis that occur in certain age groups more than others.

What are the causes of vasculitis?

Vasculitis may occur secondary to an identified underlying disease or trigger. Occasionally, an allergic reaction to a medicine may trigger vasculitis. Vasculitis can sometimes develop in conjunction with an infection. Usually in these cases, the infection causes an abnormal response in the person’s immune system, damaging the blood vessels. Viral hepatitis (a type of liver infection), is a specific infection that can be associated with vasculitis. Vasculitis may also be related to other diseases of the immune system that the patient had for months or years. For example, vasculitis could be a complication of rheumatoid arthritis, systemic lupus erythematosus, or Sjögren’s syndrome.
In many cases though, the causes of vasculitis are not known.

What are the symptoms of vasculitis?

Because any organ system may be involved, an enormous number of symptoms are possible. If the skin is involved, there may be a rash. If nerves suffer loss of blood supply, there may initially be an abnormal sensation followed by a loss of sensation. Vasculitis in the brain may cause a stroke, or in the heart may result in a heart attack. Kidney inflammation usually is not associated with symptoms and is detected by the doctor by examination of the urine. This is important to recognize as inflammation in the kidneys can lead to kidney failure unless promptly detected.

Sometimes the symptoms are non-specific. When inflammation is present in the body, we tend to respond in ways that tell us that we are not well, but those responses may not be unique to vasculitis at all. For example, along with the symptoms mentioned previously, a person with vasculitis may also have a fever or experience loss of appetite, weight loss and loss of energy.

Vasculitis_Image_3
Image Source: BMJ

Diagnosis

  • Blood chemistry tests and cell counts: Laboratory tests of blood or body fluids are performed for patients with active vasculitis. Their results will generally show signs of inflammation in the body, such as increased erythrocyte sedimentation rate (ESR), elevated C-reactive protein (CRP), anaemia, increased white blood cell count and eosinophilia. Other possible findings are elevated antineutrophil cytoplasmic antibody (ANCA) levels and hematuria.
  • Analysis of urine, both chemistry and microscopic 
  • Imaging tests such as x-ray, MRI, and CT scan, which show lung infiltrates or nodules typical of granulomatosis or vascular tissue damage
  • A CT virtual bronchoscopy can help identify stenosis and larynx problems
  • Digital subtraction angiograpy (DSA) may be useful in identifying stenosis or other abnormalities caused by vasculitis
  • Biopsy of the suspected granuloma and/or blood vessels or other affected: The definite diagnosis of vasculitis is established after a biopsy of involved organ or tissue, such as skin, sinuses, lung, nerve, and kidney. The biopsy elucidates the pattern of blood vessel inflammation.
  • Other organ functional tests may be abnormal. Specific abnormalities depend on the degree of various organs involvement.
  • An alternative to biopsy can be an angiogram (x-ray test of the blood vessels). It can demonstrate characteristic patterns of inflammation in affected blood vessels.

Vasculitis_Image_2
Image source: National Heart Lung and Blood Institute

What is the outlook for people with vasculitis?

The outlook for a person who has vasculitis will vary with the type of vasculitis that is present, what organs are being affected, how severe the vasculitis is, and how the person responds to treatment. Knowing the type of vasculitis allows the doctor to predict the likelihood of illness severity and outcome. With proper treatment, normal life spans are possible. The success of therapy is related to prompt diagnosis, aggressive treatment and careful follow-up to be sure that side effects from medications do not develop.

Once vasculitis is under control (often referred to as “remission”), medications may be cautiously withdrawn, with the hope that the patient will sustain a long remission, independent of treatment. Because some forms of vasculitis can recur (referred to as a “relapse”) after a period of remission, it is very important for patients with vasculitis to remain under the care of a knowledgeable physician.
The outcome of vasculitis is hard to predict. It will depend on the type of vasculitis you have, which organs are affected, and the severity of the condition.

If vasculitis is diagnosed early and responds well to treatment, it may go away or go into remission. "Remission" means the condition isn't active, but it can come back, or "flare," at any time.

Flares can be hard to predict. You may have a flare when you stop treatment or change your treatment. Some types of vasculitis seem to flare more often than others. Also, some people have flares more often than others.

Sometimes vasculitis is chronic (ongoing) and never goes into remission. Long-term treatment with medicines often can control chronic vasculitis, but no cure has been found. Rarely, vasculitis doesn't respond well to treatment. This can lead to disability or even death.

Ongoing care

The medicines used to treat vasculitis can have side effects. For example, long-term use of corticosteroids may lead to weight gain, diabetes, weakness, a decrease in muscle size, and osteoporosis (a bone-thinning condition). Long-term use of these medicines also may increase your risk of infection.

Your doctor may adjust the type or dose of medicine you take to lessen or prevent the side effects. If your vasculitis goes into remission, your doctor may carefully withdraw your medicines. However, he or she will still need to carefully watch you for flares.

While you're being treated for vasculitis, you'll need to see your doctor regularly. Talk with your doctor about any new symptoms and other changes in your health, including side effects of your medicines.

Common medications For treatment of vasculitis in New Zealand

Medicine
  • Steroids to control inflammation. Treatment for many types of vasculitis consists of doses of a corticosteroid drug, such as prednisone or methylprednisolone (Medrol), to control inflammation. Side effects of steroids can be severe, especially when taken over a long period of time. Side effects can include weight gain, diabetes and bone thinning (osteoporosis). You're likely to receive the lowest dose of steroids possible to control your disease. For detailed info regarding these medications you can see these links:
    Prednisone: http://www.medsafe.govt.nz/profs/datasheet/a/Apoprednisonetab.pdf
    Methylprednisone: http://www.medsafe.govt.nz/profs/datasheet/d/Depomedrolinj.pdf
    Steroids are a controlled medication and it is important that you not stop taking them without consulting your doctor.
  • Medications to control the immune system. Severe cases of vasculitis or those that don't respond adequately to corticosteroids may need treatment with cytotoxic drugs that kill immune system cells responsible for causing inflammation, in some instances they are used to help reduce the need to rely on corticosteroids. Cytotoxic drugs include azathioprine (azamun, Azasan, Imuran) and cyclophosphamide (Endoxan, Cytoxan). Another drug that helps dampen the immune system response is rituximab (Mabthera,Rituxan).
  • Researchers with America have also had some success using drugs that alter your body's immune response to treat certain types of vasculitis. Drugs that have been used, but are still being tested include mycophenolate (Cellcept), infliximab (Remicade), adalimumab (Humira) and anakinra (Kineret).
    Azathioprine: http://www.medsafe.govt.nz/profs/datasheet/a/Azamuntab.pdf
    Cyclophosphamide: http://www.medsafe.govt.nz/profs/datasheet/e/Endoxaninj.pdf
    rituximab : http://www.medsafe.govt.nz/profs/datasheet/m/Mabtherainf.pdf
  • Medications and doses vary greatly amongst patients depending on response and severity of vasculitis, so it really is important to discuss all medications and any changes to medications with your doctor.
  • It can also be a good idea to use one chemist as each prescription counts towards your total for a high user card. Check with your chemist how many scripts you have had as it can be a significant saving having a high user card for your prescriptions. There is also a new initiative known as 'Care Plus' which will be a subsidy especially for people with chronic and recurring illnesses. It will be interesting to see if Vasculitis meets the criteria though it is more likely to be on an individual case basis.
  • By sticking with the one chemist to obtain your prescriptions, your chemist may be able to advise you of any medical interactions of your medications, especially when buying over the counter medications like cold and flu medications.
  • You can also obtain a 'yellow' medication card from your GP summarising what medications you are currently taking. Keep it in a known place to your family and it can be very useful to take with you if you have to make any urgent visits to the hospital.

Emotional issues and support

Living with a chronic condition may cause fear, anxiety, depression, and stress. Talk about how you feel with your health care team. Talking to a professional counsellor also can help. If you’re very depressed, your doctor may recommend medicines or other treatments that can improve your quality of life.

Joining a patient support group may help you adjust to living with vasculitis. You can see how other people who have the same symptoms have coped with them. Talk with your doctor about local support groups or check with an area medical centre.

Support from family and friends also can help relieve stress and anxiety. Let your loved ones know how you feel and what they can do to help you.

Tips for patients with vasculitis

  • Each case of vasculitis is different – Get to know your disease. Educate yourself, the more knowledge you have, the more you will be able to participate in the management of your disease. Be your own advocate - you know your body; don't be afraid to speak up. Vasculitis is rare and not as well known as it should be, be persistent ask questions.
  • Build an honest and open relationship with your doctor. It takes a team to get you on the right track and that includes you. Your doctor is only human - they need to understand that we are too complex for one doctor to be able to understand, support and handle it all. Bring in the cavalry - specialists are called that for a reason - they deal with specifics. If you are not satisfied with an answer, demand a second opinion. It's your life after all!
  • Do not play with medications! Learn about them, the purpose for taking them and the side effects that you might encounter. Discuss this with your doctor and pharmacist.
  • Be honest with yourself and others. Pace yourself, “Fine” is not always your best answer. It’s okay to say you’re having an “Off” day.
  • Ask for help. Call in the Calvary when necessary. Have people in place to help with child care, cleaning, groceries etc. It’s okay to ask for help, it also makes others feel like they can finally do something for you. Let them.
  • It is normal to grieve the loss of the “old” self. Learn to come to terms with your disease and not deny it. Try and find something that has been positive about the whole thing.
  • Give yourself permission to be depressed for short periods of times. Its okay, you are going through a big adjustment. Talk to someone like a therapist or close friend. Find your sense of humour and develop a positive attitude, and then get on with living!
  • Don’t lie around. Make sure you get out of bed and dressed each day even if you only make it to the couch. Try to do your hair and put on some make-up, it gives you a better sense of well being. Move around even if you hurt. Try getting up and walking around may help relieve your pain.
  • Pamper yourself! This is your chance to let others take care of you. Let them and don’t feel bad about it. Treat yourself to a massage or have someone give you one.
  • Make lifestyle changes. Eat balanced, low fat meals and get regular exercise. Try yoga or meditation to ease muscle tension. Get enough sleep, this will help your body rejuvenate itself. Reduce your stress and anxiety because this may also cause fatigue.
  • Keep a journal of symptoms, medication dosages and their side effects, plus your feelings. It will come in handy each time you visit your doctor.
  • Plan a night out. Plan a friend’s night out to get your mind off of your illness.
  • Seek a support group. It is great to talk to others who are going through similar experiences.

And remember…You are not alone!