Vasculitis

We do not know what causes most types of vasculitis. Genetic factors (different genes) appear be somewhat important in the disease. Vasculitis is thought to be an autoimmune disease, which means the body comes under attack by its own immune system. In vasculitis, the immune system attacks blood vessels. Some cases of vasculitis are caused by reactions to medicines. Also, some chronic (long-term) infections, including with hepatitis C or hepatitis B virus, can cause vasculitis.

Vasculitis can be a part of other rheumatic diseases, mainly including systemic lupus erythematosus, rheumatoid arthritis and Sjögren’s syndrome. Most patients with vasculitis have none of these diseases.

Physicians suspect vasculitis when a patient has symptoms and abnormal results of the physical exam, lab tests or both, and there is no other clear cause. The most common tests are:

• Biopsy—surgical removal of a small piece of tissue for inspection under a microscope
• Angiography—a type of X-ray to look for abnormalities of blood vessels
• Blood tests

For most patients, doctors can detect the type of vasculitis based on the size of the affected blood vessels (see Table) and the organs involved. To find small-vessel vasculitis, doctors most often do a biopsy, such as of the skin or a kidney. Detection of medium-vessel vasculitis happens by either biopsy (for instance, of skin, nerve or brain) or angiography. Angiography also is the test that often finds large-vessel vasculitis. Detecting giant cell arteritis often involves a biopsy of an artery in the scalp.

A few forms of vasculitis, such as Behçet’s disease and Kawasaki disease, are usually detected on the basis of a collection of clinical findings rather than biopsy or angiography.

Some blood tests are so suggestive of a certain type of vasculitis that a positive (abnormal) test can be enough evidence to help doctors make the diagnosis. The most useful of these tests is for “antineutrophil cytoplasmic antibodies”—often referred to as ANCA. A positive ANCA test can help detect these types of vasculitis: granulomatosis with polyangiitis (Wegener’s), microscopic polyangiitis or eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Other lab tests can show damage to organs, but the tests are not enough to prove vasculitis.

Largest arteries: aorta and major branches

• Giant cell arteritis
• Takayasu’s arteritis
• Aortitis in Cogan’s syndrome
• Aortitis in spondylarthropathies
• Isolated aortitis

Medium-sized arteries

• Kawasaki disease
• Polyarteritis nodosa

Small and medium-sized arteries

• ANCA-associated vasculitis
• Granulomatosis with polyangiitis (former name: Wegener’s granulomatosis)
• Microscopic polyangiitis
• Eosinophilic granulomatosis with polyangiitis (Churg-Strauss )
• Primary angiitis of the central nervous system

Small arteries

• IgA Vasculitis (Henoch-Schönlein)
• Vasculitis related to rheumatoid arthritis, systemic lupus erythematosus and Sjögren’s syndrome
• Cryoglobulinemic vasculitis
• Anti-GBM disease (Goodpasture’s)
• Drug-induced vasculitis

Arteries and veins of various sizes

• Behçet’s disease
• Relapsing polychondritis

Glucocorticoids: Glucocorticoids (prednisone, prednisolone or others), often referred to as “steroids,” are an important part of treating most forms of vasculitis. The dose and length of treatment depend on how bad the disease is and how long the patient has had it. These drugs help reduce inflammation but can have long-term side effects.

Other drugs: Doctors sometimes prescribe immune-suppressing drugs because their long-term side effects may be less serious than those of glucocorticoids. This is called “steroid-sparing” treatment. Cyclophosphamide is one of the strongest of these drugs, and doctors may prescribe it when severe disease endangers vital organs.

For less serious vasculitis, patients may receive methotrexate, azathioprine or other immune-suppressing drugs. Doctors often prescribe these drugs to treat other rheumatic diseases, but they are useful for vasculitis, too.

Newer drugs designed to treat other autoimmune and inflammatory diseases may also help vasculitis. Researchers found that one of these drugs, rituximab effectively treats severe cases of certain forms of vasculitis. These include granulomatosis with polyangiitis, microscopic polyangiitis and cryoglobulinemic vasculitis. Some patients with the most severe cases of these diseases may receive plasma exchange (“plasmapheresis”) or intravenous immunoglobulin (often called “IVIg”).

Surgery: Damage from severe vasculitis sometimes requires surgery. This may involve vascular bypass grafting (a surgery to redirect blood flow around a blockage in a blood vessel). Depending on where the damage is, other possible operations are sinus surgery or a kidney transplant.

Vasculitis can be short term or lifelong. Doctors often focus, with good reason, on preventing permanent damage to vital organs (such as the lungs, kidneys and brain) and the nerves. It is crucial, of course, to prevent death and long-term disability from vasculitis. Yet, other issues often trouble patients. These include fatigue (feeling very tired), pain, arthritis, nose and sinus problems, and many other problems.

Side effects from medications, especially glucocorticoids, also can be troubling. Patients taking immunosuppressants are at risk of infections. Follow your doctor’s advice on how to reduce your infection risk.

Fortunately, with current treatments, the outcome for patients with vasculitis is often good.

Rheumatologists are usually the specialists with the most overall knowledge about vasculitis. Thus, they direct the care of patients, particularly those with chronic or severe disease. Patients with vasculitis often benefit from seeing experts in the organ systems that are or might become affected. Doctors that patients may need to see include a dermatologist (skin doctor), neurologist (expert in nervous system diseases), ophthalmologist (eye doctor), otorhinolaryngologist (ear, nose and throat doctor), nephrologist (kidney doctor) or pulmonologist (lung doctor).