What is vasculitis?

Vasculitis is an autoimmune disorder that occurs when the immune system attacks the blood vessels, creating inflammation and damage to the organs those vessels supply. Blood vessels can become narrowed, scarred, weakened, or blocked, resulting in a lack of blood flow, with potentially serious consequences.

There are many types of vasculitis, each with specific symptoms. Some are chronic and can result in damage, and others have symptoms that may come and go.

Vasculitis is rare, but can affect anyone. Symptoms may be mild and affect one organ such as the skin, or it may involve several organs.

General Signs and symptoms of vasculitis include:

  • Fevers
  • Weight loss
  • Headaches
  • Fatigue
  • Rashes
  • Numbness or weakness

Selected Types of Vasculitis:

Behcets Disease

This disease can cause inflammation in the arteries and veins. Signs and symptoms include oral and genital ulcers, eye inflammation and skin lesions.

Buerger’s Disease (thromboangiitis obliterans)

This disease can cause inflammation and clots in the blood vessels that supply your hands and feet. It can cause pain in the extremities and ulcers on your fingers and toes. This disorder is associated with cigarette smoking

Cryoglobulinemic Vasculitis

This disease can results from abnormal protein in the blood. It can be associated with infectious diseases such as Hepatitis C and also with underlying blood cancers. Signs and symptoms include rashes, joint pain, numbness, and weakness.

Eosinophilic Granulomatosis with polyangiitis (Churg-Strauss)

This condition is associated with increased eosinophils (a type of white blood cell) and can affect the kidneys, lungs and nerves. It is often associated with asthma

Granulomatosis with Polyangiitis (Wegeners or GPA)

This is another one of the ANCA-associated vasculitides. It can affect the sinuses, upper airway (trachea), lungs, kidneys and many other organs. Patients can experience joint pains, eye inflammation or redness, cough, blood in the urine, hearing or vision problems, rashes, as well as other symptoms of fatigue, weakness and fevers.

Henoch-Schonlein purpura (HSP)

This condition usually affects children, but can occur at any age. It results from inflammation in the capillaries (the smallest blood vessels) and can cause damage to the skin, joints, bowel and kidney. Signs and symptoms include abdominal pain, blood in the urine, joint pain, and a rash on your buttocks or lower legs.

Kawasakis Disease

This disease affects young children and is associated with high abrupt fevers, redness in the eyes, rashes, swelling of the hands and feet, swollen glands in the neck, and redness/cracking of the tongue and lips. It has a good prognosis with treatment, but in some cases can lead to heart abnormalities.

Microscopic Polyangiitis (MPA)

This disease can affect individuals from any ethnic background at any age, but typically begins in middle-aged people. It is one of the “ANCA-associated vasculitides”. Signs and symptoms can include fevers, weight loss, kidney inflammation with blood in the urine, nerve damage and skin lesions

Polyarteritis Nodosa (PAN)

This type of vasculitis affects the medium and small sized arteries. It can result in thinning of blood vessel wall that result in aneurysms (outpouchings of the blood vessels). Some of the symptoms include weight loss, abdominal pain, nerve and motor problems, testicle pain, and skin ulcerations or nodules

Takayasu’s Disease

This is a type of vasculitis affecting the large arteries in the body (the aorta and its branches).  It classically affects young women, and can be associated with high blood pressure in the body or in the blood vessel supplying the lungs. The disease can lead to narrowing and blockages or areas of weakness with dilation or aneurysms of the blood vessel. Symptoms include fatigue, weight loss, low grade fevers, arm or chest pain, decreased pulses, and possibly heart failure.

Temporal Arteritis (Giant Cell Arteritis)

This condition usually affects older individuals (>50) and is associated with severe headaches. It can also cause scalp tenderness, jaw pain, changes in vision. If it is left untreated, it can lead to permanent vision changes and blindness. This disease is associated with polymyalgia rheumatic (PMR), which causes pain and soreness in the muscles of the shoulders/neck and hips.

When should I see a doctor?

It is important to diagnose vasculitis early to control inflammation and prevent damage, so you should make an appointment if you have any worrisome signs or symptoms. If your primary doctor suspects a vasculitis, it is important to establish care with a Rheumatologist for further workup.

What types of tests should I expect to have done?

Depending on the signs and symptoms you are experiencing, the workup for underlying vasculitis can include blood tests, urine tests, xrays or other imaging studies, electrocardiogram (EKG), lung function tests, nerve conduction studies, and biopsy if needed.

If I have vasculitis, what happens next?

We will work with you to try and make an accurate diagnosis as quickly as possible. Depending on the type of vasculitis and severity of the disease, you may need to see other specialists to help co-manage your symptoms.

Based on the type of vasculitis you have, the severity of the disease and the organs affected, we will discuss what treatment(s) are needed. This could include medications to help stop inflammation and control your immune system, lifestyle modifications (diet, exercise, stress reduction, tobacco avoidance), preventative care (immunizations, lab monitoring and precautions), and possibly surgical interventions if needed.