Vasculitis

Vasculitis is a group of disorders that cause inflammation of the blood vessels.

The inflammation may affect blood vessels of any size and anywhere in the body. Vasculitis may affect either arteries, veins or capillaries (very tiny blood vessels).

There are two different types of vasculitis depending on the distribution:

• Focal: It affects only a single location within a blood vessel.
• Widespread: There are different areas of inflammation in the same organ, tissue or even affect more than one organ.

In most cases, vasculitis causes are unknown, but it is clear that it is related to an autoimmune response that attacks healthy cells in the body.

Some types of vasculitis are due to an allergic response to specific substances (for example, after taking a drug) or to a virus (hepatitis B or hepatitis C).

Many autoimmune diseases have vasculitis as one of their complications:

• Lupus (Systemic Lupus Erythematosus)
• Rheumatoid arthritis
• Scleroderma
• Polymyositis
• Polyarteritis nodosa
• Kawasaki's disease
• Henoch-Schönlein purpura
• Temporal arteritis (also called giant cell arteritis)
• Takayasu's arteritis
• Serum sickness
• Wegener's granulomatosis
• Buerguer's disease
• Behcet's disease
• Sjögren syndrome
• Eosinophilic granulomatosis with polyangiitis (EGPA)

Vasculitis can affect people at any age:

• Behcet's disease and Takayasu's arteritis are more common in young people (20 to 30 years old).
• Buerguer's disease, polyarteritis nodosa and Wegener's granulomatosis are a middle-aged adult disease (40-60 years old).
• Kawasaki's disease mainly affects children and teenagers.
• Temporal arteritis (giant cell arteritis) mainly affects elderly people.

Symptoms of vasculitis depend on the severity of the inflammation produced.

In mild vasculitis, the only symptoms are small reddish-purple dots (called petechiae) on the skin due to tiny amounts of blood seeping out of leaky blood vessels.

If vasculitis is widespread, there may be more symptoms such as:

• Fever
• Muscle and joint pain
• Decreased appetite and weight loss
• Fatigue

The most affected parts of the body are:

• Skin: It may appear rashes, bumps under the skin, petechiae or larger reddish-purple circles (purpura).
• Joints: There may be pain or inflammation in the joints, resulting in arthritis.
• Nervous system: Inflammation in a brain vessel may cause headaches. If the affected vessel is related to the eyes, vision may become disturbed. Nerves involved in the extremities may result in weakness.
• Gastrointestinal system: Abdominal pain, vomiting, and diarrhea.
• Lungs: Shortness of breath, chest pain and wheezing (high-pitched whistling sound made while breathing).

The inflamed vessels can block the flow of oxygen to certain organs, causing severe complications such as:

• Gangrene
• Stroke (Cerebrovascular accident CVA)
• Aortic aneurysm or heart attack
• Kidney failure

Diagnosis of vasculitis involves detecting the presence of the inflammatory process.

In a blood test, erythrocyte sedimentation rate (ESR) will be over the normal range, there will be anemia and also an increase in the white blood cells (WBC) count. ANCA testing (antineutrophil cytoplasmic antibody) is commonly used when vasculitis is suspected.

The definitive diagnosis is to perform an angiography, an X-ray procedure that involves injecting dye into a major artery and then taking X-ray pictures to examine the blood vessels.

Even though there are many different types of vasculitis with many organ systems affected, treatments are essentially the same in all cases.

They all involve trying to decrease the activity of the immune system using corticosteroids (like Prednisone) to interfere in the inflammatory process.

Cyclophosphamide is also used for severe cases of vasculitis.

The prognosis for vasculitis is quite variable.

In cases related to reactions to drugs, they may resolve with the suppression of medication with no further treatment.

Temporal arteritis, serum sickness, Henoch-Schonlein purpura, and Kawasaki's disease (not affecting the heart) usually have an excellent prognosis.

Polyarteritis nodosa and Wegener's granulomatosis have high rates of fatal complications.