Vasculitis refers to a group of diseases in which inflammation of the blood vessels is the hallmark feature. Such inflammation can cause narrowing and weakness of the vessel lining, and in some instances a tendency to form small clots in the affected vessels. This can result in damage to the tissues or organs being supplied by those blood vessels, including the kidney, lung, skin, nerves, or even the brain.
Patients with vasculitis may also have pain and fever because of the systemic inflammation. Blood vessels throughout the body, including the major veins and arteries may be affected and damage to one or more organs may occur. The severity of the condition ranges considerably from mild cases to those that are disabling or even life threatening.
Vasculitis can occur spontaneously as a disease unto itself, or in other instances can occur in the context of a broader autoimmune disease such as rheumatoid arthritis or lupus. In those autoimmune diseases, the body perceives its own tissue as "foreign" and the immune system attacks the body’s own cells. Vasculitis may also be a reaction to certain medications or in the presence of certain chronic infections.
Symptoms of vasculitis are related to the part or parts of the body affected. For example, vasculitis affecting the vessels close to the skin’s surface are characterized by rash, whereas forms of the disease that affect blood vessels that supply the nerves may cause alterations in sensation. Treatment for vasculitis may involve monitoring in mild cases, but prompt intervention with immunosuppressant therapy may be required in patients with more aggressive forms of condition. Accurate diagnosis by a rheumatologist is essential as these are generally very treatable diseases as long as the diagnosis is made before significant organ damage occurs.
The most common types of vasculitis in children are Henoch-Schonlein purpura (also known as IgA vasculitis) and Kawasaki disease. Other types of vasculitis include polyarteritis nodosa, Takayasu arteritis, ANCA-associated vasculitis, Behcet’s disease, and primary vasculitis of the central nervous system. All of these diseases are very uncommon in childhood.
The symptoms a child has depend on the specific type of vasculitis and which blood vessels are affected. Many types of vasculitis have general symptoms such as prolonged fevers, fatigue, and weight loss. Children may have a characteristic rash that looks like small red dots or bruising (petechiae or purpura), or ulcers in their mouth or on their genitals. In children with kidney involvement, urine changes and high blood pressure may occur, and if the nervous system is affected, the child may have seizures, stroke, or other neurological changes. Other concerning symptoms include trouble breathing, coughing up blood, or bloody diarrhea. If you are ever concerned for your child’s immediate safety, it is important to bring him or her to the emergency department at once for evaluation.
Vasculitis can be challenging to diagnose since it has a wide variety of symptoms that are seen in many other disorders. Blood, urine and stool tests may be helpful to confirm the presence of inflammation, and to rule out other underlying causes. Some children with vasculitis have a positive autoantibody (antibody against the self) called ANCA, which may help guide diagnosis and treatment. In some cases, imaging of the blood vessels using a technique called angiography can be useful. Your child’s doctor may also recommend a biopsy of a small piece of tissue, such as the skin, to look closely for blood vessel wall inflammation.
Some types of vasculitis can be treated with daily oral medicines, while other types require IV medication. The main goal of treatment is to control the symptoms as soon as possible (induction), and then maintain long-term control (remission). This is typically done using a combination of steroids and other immunosuppressive drugs. Depending on the manifestations of the disease, children may also require other types of medication, including antihypertensives for blood pressure control.