Vasculitis - What You Need to Know

Vasculitis is Sometimes Associated with Other Rheumatic Diseases

A.D.A.M.

Vasculitis is a rare condition that involves inflammation of the blood vessels. Vasculitis develops when your immune system attacks your own blood vessels. Any of the body's blood vessels—arteries (which carry blood away from your heart), veins (which carry blood to your heart), and capillaries (which connect small arteries and veins)—can be affected. Inflammation of a blood vessel may cause it to narrow or close off, which limits or prevents normal blood flow.

Rarely, the affected blood vessel can stretch and weaken, causing an aneurysm.

Causes of Vasculitis

The cause of vasculitis is not completely known or understood. It is believed that infection, medication reactions, and autoimmune disease can be triggers. Vasculitis can be associated with other rheumatic diseases, such as lupus, rheumatoid arthritis, and Sjogren's syndrome—but most patients with vasculitis do not have any of these underlying conditions. Depending on the cause, vasculitis can be mild, severe, or life-threatening. It can occur as a single episode of symptoms or multiple events.

Signs and Symptoms of Vasculitis

The signs and symptoms linked to vasculitis vary, depending on the type of blood vessel and organs involved. Common signs and symptoms include fever, loss of appetite, weight loss, fatigue, and pain. Signs and symptoms that are more related to the affected body part include:

  • Skin - reddish/purple spots, nodules, or ulcers
  • Lungs - shortness of breath, coughing
  • Nerves - numbness or weakness in an extremity
  • Joints - aches, pains, and arthritis
  • Gastrointestinal tract - mouth ulcers, abdominal pain, intestinal bleeding
  • Sinus and ENT - sinus inflammation, middle ear inflammation, nose ulcers, hearing loss
  • Eyes - red, itchy, or burning eyes, blurry vision, light sensitivity, blindness
  • Brain - headache, mental function changes, stroke-like symptoms

Diagnosis of Vasculitis

As with most medical conditions, the diagnosis of vasculitis is based on a combination of findings that include signs and symptoms, medical history, physical examination, and results from diagnostic tests. Certain blood tests can point to signs of anemia (hemoglobin and hematocrit tests) and non-specific signs of inflammation (erythrocyte sedimentation rate and C-reactive protein). Another blood test, ANCA (anti-neutrophil cytoplasmic antibodies), is used as a diagnostic marker for systemic vasculitis.

Blood pressure can be elevated, especially when there is kidney involvement. High levels of protein or blood cells found in a urinalysis can also be indicative of kidney involvement. Electrocardiogram (EKG) or heart echocardiogram is used to check any abnormalities related to the heart. Chest x-ray, lung function tests, abdominal ultrasound, CT scans of organs, and MRI angiography to check blood vessels are all tests available to help formulate a diagnosis.

Examining tissue samples via biopsy is another means for checking blood vessels for signs of inflammation or tissue damage.

Treatment of Vasculitis

The prescribed treatment will depend on the type of vasculitis and the organs involved, as well as the severity and progression of the condition. Typically, medications will be prescribed to control inflammation, immunosuppressants may be used to treat the overactive immune system, and other medications can help mitigate any specific symptoms. Glucocorticoids, more commonly referred to as steroids, are a mainstay in the treatment of vasculitis.

Types of Vasculitis

There are about 20 different conditions that are classified as vasculitis, including:

The Bottom Line

While vasculitis is considered a rare condition, there is an urgency related to its diagnosis. There is no cure for vasculitis, but early diagnosis and treatment can help control symptoms and manage the progression of tissue and organ damage.

Sources:

Paul A. Monach, MD, PhD and Peter A. Merkel, MD, MPH. Vasculitis. American College of Rheumatology Patient Education. Updated May 2015.

What Is Vasculitis? Johns Hopkins Vasculitis Center. Johns Hopkins Medicine.

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