Vasculitis

Vasculitis refers to a group of infections that destroy blood vessels. The exact disease is classified based on the extent of the disease and the type of blood vessels involved. Some of the diseases are common among children, and others are rarer.

The disease is not hereditary; most likely the disease develops as a result of a number of different factors (infections, genetic, environmental). The diseases are not contagious.

For a list of several types of Vasculitis, See bottom.

What happens to the blood vessels in Vasculitis?
The child’s immune system attacks the walls of the blood vessels, causing them to become infected.

The inner layer of the blood vessel plays an important role in the process. For healthy people, this layer helps keep the blood flow normal. However, when inflamed, the blood will clot, causing blockages. The diseased cells become a part of the normal blood flow and travel to other areas of the body, causing other areas to become infected. An infected wall is more easily penetrated, enabling blood to leave the blood vessels – causing swelling and edema. Biopsies and angiographies will indicate changes to the blood vessels caused by the infection.

A low supply of blood can cause damage to different tissues and systems in the body. If the infection affects a blood vessel that supplies blood to a vital organ in the body – the child can suffer from serious health repercussions. A multi-system infection also causes fever, weakness and drowsiness. The symptoms depend on the type of vasculitis.

Diagnosis of Vasculitis
Diagnosing the disease is not so simple. The symptoms are similar to symptoms of other, more common, childhood diseases. In order to make a proper diagnosis, the doctor must check each of the child’s symptoms, blood tests, urine samples and imaging results. Biopsies may be required in some cases. The disease is relatively rare; so many cases will require your child to be transferred to a medical center with a rheumatologist.

In most cases, the disease is brought under control.

Treatment Methods for Vasculitis
Treating vasculitis is a complex process and takes a long time. The goal is to control the disease as well as prevent complications and side effects. The first phase of treatment generally includes a high dose of corticosteroids with strong immunosuppressents. Afterwards, the doctor will prescribe different drugs to prevent and stop the infection. If the regular medications don’t work the doctors will use biological medications. Prolonged use of corticosteroids can lead to osteoporosis, which can be prevented with a calcium supplement. Sometimes, the child will also need physical therapy or medication for high blood pressure.

Monitoring the Disease
The doctor will evaluate the progression of the disease in addition to hte effectiveness of treatment and the treatment side effects. This way the doctor will be able to prescribe the best possible treatment for the patient. Monitoring and the frequency of doctor visits are dependent on the severity of the disease. The frequency of the doctor visits will decrease as the disease gets better.

There are a number of ways to evaluate the progression of the disease. Parents are requested to report any differences in their child’s condition. In some cases, blood pressure or urine need to be checked. A thorough physical examination and your child's physical complaints are extremely important in properly evaluating the disease’s progression. The blood tests and urine samples help doctors evaluate the severity of the infection, changes in organ function and the side effects of the medications. Sometimes, the doctors may require further testing.

How long does the disease last?
Rare primary infections of the blood vessels are usually prolonged, and may last throughout the person’s entire life. In some cases, they start as severe, even life threatening diseases, and slowly become chronic and less severe.

What is my child’s long-term prognosis?
Every case of primary vasculitis is different. The prognosis depends on the type and amount of blood vessels involved, the amount of time that passed from the disease’s onslaught to the beginning of treatment, and how long it takes the child to react to treatment. The risk of damage to the body’s organs depends on the severity of the infection. Damage to the body can cause lifelong repercussions.

In most cases, your child won’t experience vasculitis outbreaks after the first year of treatment. There are cases where children do not experience any more outbreaks for the rest of their lives after the first round of treatment, but in most cases, your child will require prolonged treatment and experience several outbreaks which require more aggressive treatment. If the disease goes untreated, it can be fatal. Since the disease is so rare, it is hard to know the long-term prognosis.

Living with Vasculitis
The child’s life (and subsequently the parents’ lives) is in turmoil before a proper diagnosis and treatment. Once treatment begins and the disease is brought under control, the child’s life will return to normal. It is important to encourage your child to return to school after the infection is under control. The teachers should be made aware of his situation. When the child isn’t fighting an outbreak, encourage him to get involved in sports and other activities.

There is no proof that a specific diet influences the course of the disease. The child should eat a balanced diet with the recommended amount of protein, calcium and vitamins. Avoid junk food, sodium and fats during corticosteroid treatment.

Cold weather may exacerbate blood flow to your child’s hands if there are already problems due to the infection.

If your child develops an infection while on immunosuppressants, it will most likely manifest more severely. If you or another family member is exposed to an infection (even a common one, such as the chicken pox), you should get inoculated immediately. In cases of infection, your child will be given an antibiotic treatment in order to prevent a pneumonia (which is particularly severe among patients with compromised immune systems).
You should not have your child inoculated against measles, rubella or tuberculosis when they are on immunosuppressants.

What About My Child's Future - Pregnancy, Sex Life, etc.?
It’s important to avoid pregnancy while experiencing an outbreak, since most of the medications can harm a fetus. Some drugs may affect a person’s reproductive system. This depends on the accumulative dose of the drug and is very rare among children and adolescents.