Treatments for Vasculitis in Children

What are the treatment options for vasculitis?

In some children, certain forms of vasculitis will improve on their own — for instance, Henoch-Schönlein purpura may run its course in four to six weeks with no treatment beyond bed rest, plenty of fluids and, occasionally, nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen. But other children will need medications to control their vasculitis and head off or treat serious complications.

Medications commonly used to treat vasculitis include:

  • Corticosteroids -- often prednisone -- are not the same as the anabolic steroids that athletes sometimes take. These are extremely powerful, fast-acting drugs that fight inflammation by suppressing the entire immune system. However, doctors work to phase them out as soon as possible because of their potential side effects, which can include high blood pressure and reduced bone density.
  • Steroid-sparing immunosuppressants offer many of the benefits of corticosteroids -- and sometimes with fewer side effects -- but take longer to work. The most powerful of this group is cyclophosphamide (Cytoxan); others include azathioprine (Imuran); methotrexate;and mycophenolate mofetil, or MMF (CellCept).
  • Biologics are a relatively new class of steroid-sparing therapies based on compounds made by living cells. Instead of suppressing the entire immune system, biologics are more like smart bombs -- they only target certain parts of it. Biologics now being used for vasculitis include tumor necrosis factor (TNF) blockers such as infliximab (Remicade). Side effects vary by medication, but are generally milder than with other kinds of steroid-sparing therapies.

Children with some kinds of vasculitis — particularly Kawasaki disease — may also benefit from IVIg (intravenous immunoglobulin). This is a blood product made up of healthful antibodies that is delivered by IV, and can help get the immune system back on track without suppressing its normal function.

In rare instances, children with vasculitis will develop complications that require surgery — for instance, if an abnormal bulge (aneurysm) develops in a blood vessel wall, doctors may need to operate to keep it from rupturing.

What is the long-term outlook for children with vasculitis?

What the future may hold for your child will depend a good deal on what kind of vasculitis he has. Kawasaki disease, for instance, tends to be what doctors call “self-limiting” — the illness usually runs its course in a month or two and does not recur. However, there is a lot of variation even within the individual types of vasculitis, depending on how severe the disease is and what organs are involved. Looking at the example of Kawasaki again, if there is serious inflammation in the coronary arteries, it may cause permanent heart damage.

In all kinds of vasculitis, doctors aim to quench the inflammation and achieve remission — that is, the disease becomes completely “quiet,” with no signs or symptoms. Once the vasculitis is under control, they may then cautiously withdraw medications with the hope that the disease will stay in remission.

Your child’s doctor will be the best source of information on the likely long-term outlook for your child. However, if diagnosed and treated before any serious complications have occurred, the vast majority of children with vasculitis will achieve remission and go on to lead full and normal lives.