Dr. Anu Daber

Dr Anu Daber Best Rheumatologist (Gold Medalist) EX- Rheumatologist (AIIMS, New Delhi) at Arthritis and Rheumatology Clinic in Gurgaon

Systemic Vasculitis

What is Vasculitis?

Vasculitis refers to inflammation of the blood vessels. These vessels include arteries and veins. Vasculitis can result in poor blood flow to tissues throughout the body, such as the lungs, nerves and skin. Thus, vasculitis has a wide range of signs and symptoms (what you see and feel), such as:

  • Shortness of breath and cough
  • Numbness or weakness in a hand or foot
  • Red spots on the skin (“purpura”), lumps (“nodules”) or sores (“ulcers”)

On the other hand, vasculitis of the kidneys may produce no symptoms at first but is still a serious problem due to renal failure. Vasculitis can be mild or disabling, or even lead to death. Patients can have one episode of vasculitis or have repeated episodes over several years. Most types of vasculitis are rare.

What causes Vasculitis?

We do not know what causes most types of vasculitis. Genetic factors (different genes) appear to 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 hepatitis C or hepatitis B virus, can cause vasculitis. Vasculitis can be a part of other rheumatic diseases, mainly systemic lupus erythematosus, rheumatoid arthritis and Sjögren’s syndrome. However, most patients with vasculitis have none of these diseases.

Who gets Vasculitis? Vasculitis affects persons of both sexes and all ages. A few forms of vasculitis affect certain groups of people. For instance, Kawasaki disease occurs only in children. IgA Vasculitis (Henoch-Schönlein purpura) is much more common in children than adults. On the other hand, giant cell arteritis occurs only in adults over 50 years old.

How is Vasculitis diagnosed?

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 detecting autoantibodies

For most patients, doctors can detect the type of vasculitis based on the size of the affected blood vessels 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 (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. 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.

Types of Vasculitis:

Largest arteries: aorta and major branches Giant cell arteritis Takayasu’s arteritis Aortitis in Cogan’s syndrome
  Aortitis in spondyloarthropathies Isolated aortitis  
Medium-sized arteries Kawasaki disease Polyarteritis nodosa
  ANCA-associated vasculitis Granulomatosis with polyangiitis (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, SLE and Sjögren’s syndrome Cryoglobulinemic vasculitis Anti-GBM disease (Goodpasture’s syndrome) Drug-induced vasculitis
Arteries and veins of various sizes Behcet’s disease Relapsing polychondritis

Abbreviations: ANCA, antineutrophil cytoplasmic antibodies; GBM, glomerular basement membrane. Doctors can detect the type of vasculitis in most patients with the disease based on the size of the affected blood vessels and damage to the organs most often involved.

How is Vasculitis treated? 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 side effects may be less serious than those of glucocorticoids. This is called “steroid-sparing” treatment. Cyclophosphamide is 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 (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.

Living with Vasculitis 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. Side effects from medications, especially glucocorticoids, also can be troubling. Patients taking immunosuppressants are at an increased 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.

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