Vasculitides Health Article

When to call the doctor

Although not all children who are eventually diagnosed with vasculitis will have all of the following signs and symptoms, parents should consult the doctor if most are present:

• The child's symptoms are constitutional; that is, they affect his or her overall physical health on a basic level. Malaise (a general feeling of physical discomfort), loss of appetite, fever, and loss of energy are examples of constitutional symptoms.
• The symptoms involve more than one organ or organ system.
• The child has noticeable purpura.
• The child has tingling or other unusual sensations followed by numbness in certain parts of the body.

Diagnosis

The diagnosis of vasculitis is complicated by several factors. To begin with, many of the early symptoms of the childhood vasculitides are not specific to these disorders and may have a wide range of other possible causes. In many cases the doctor may not be able to make the diagnosis until specific organs or organ systems are affected. The doctor will begin by ruling out such other possible diagnoses as bacterial or viral infections, collagen vascular disease, hypersensitivity reactions, and malignant tumors.

Another factor that complicates diagnosis is that the various childhood vasculitides have overlapping symptom profiles. Although lists of diagnostic criteria have been drawn up for the various disorders in this group, some patients do not meet the full criteria of any one disorder.

The first step in diagnosis is taking a careful history. The child's doctor may be able to narrow the diagnostic possibilities on the basis of the patient's age, sex, ethnicity, and a history of recent vaccinations or upper respiratory infections. The doctor will also ask whether the child is experiencing fever, abdominal cramping, diarrhea, or pains in the muscles and joints. The second step is a general physical examination. Several of these disorders affect the circulation or eyes as well as the skin. For example, Takayasu arteritis affects the patient's pulse and blood pressure, as well as producing small hemorrhages in the retina of the eye, while Kawasaki disease is characterized by conjunctivitis (inflammation of the tissues lining the eyelids). The doctor will examine the child's skin for purpura, other skin rashes or ulcers, reddening or swelling of the skin, and will note the locations of these abnormalities. In most cases the doctor will refer the child to a specialist for further evaluation. The specialist may be a pediatric rheumatologist, cardiologist, neurologist, or specialist in infectious diseases.

Laboratory tests for vasculitis include blood and urine tests. The blood tests include a complete blood count, a blood serum chemistry panel, erythrocyte sedimentation rate (ESR), tests for rheumatoid factor and circulating immune complexes, and tests for antineutrophil cytoplasmic antibodies (c-ANCA and p-ANCA). Urinalysis is done to evaluate kidney function. The doctor may also order skin, muscle, lung, or sinus biopsies in order to distinguish among the various childhood vasculitides.

Imaging studies that are used to diagnose the various childhood vasculitides include chest x rays or CT scans of the sinuses (Wegener's granulomatosis); CT scan of the aorta, angiography or ultrasonography (Takayasu's arteritis); arteriography or echocardiography (IPAN); chest x ray and echocardiography (Kawasaki disease); chest x ray, abdominal ultrasound, or barium contrast study of the digestive tract (Henoch-Schönlein purpura).

Treatment

The treatment of children with one of these disorders is highly individualized; it is tailored to the specific organs affected and the child's overall condition. Henoch-Schönlein purpura usually resolves on its own without any specific therapy. The general goals of treatment for vasculitis are to reduce inflammation in the affected blood vessels; maintain an adequate blood supply to the vital organs and skin; and monitor the side effects of the medications given to treat vasculitis.

Medications

Most patients with vasculitis will be given corticosteroids (usually prednisone) to reduce joint pain if present and inflammation in the blood vessels. Other types of drugs that are commonly used are the immunosuppressants (usually cyclophosphamide, methotrexate, azathioprine, or etanercept) and anticoagulants (usually heparin). Immunosuppressants are drugs that are given to treat inflammation by lowering the intensity of the body's reaction to allergens and other triggers, while anticoagulants are given to prevent blood clots from forming and blocking blood vessels that have already been narrowed by inflammation. Children with muscle or joint pain may be given nonsteroidal anti-inflammatory drugs, or NSAIDs, many of which are available without a prescription. An antibiotic (usually trimethoprim-sulfamethoxazole) is sometimes given to children with Wegener's granulomatosis to control flares, or recurrences of the disease.