Patients

Living with Vasculitis

Being diagnosed with a chronic medical condition can be draining physically and psychologically. It’s not just the patients who suffer. Caretakers, families, and friends are also affected.

Coping with vasculitis is life-altering and changes every aspect of daily life.

What is Vasculitis?

Vasculitis is a general term that refers to inflammation of the blood vessels. It is used to describe a family of nearly 20 rare diseases, characterized by narrowing, weakening or scarring of the blood vessels, which can restrict blood flow and damage vital organs and tissues.

Vasculitis can affect any of the blood vessels of the body, including arteries, veins and capillaries. Symptoms depend on the organs and tissues affected, and can vary from person to person. Some forms of the disease are mild and may improve on their own, while others involve critical organ systems and may require lifelong medical care. Early diagnosis and treatment are extremely important to avoid potentially life-threatening complications.

It is common for people with vasculitis to experience periods of relapse and remission, so regular doctor visits and follow-up monitoring are recommended. Proper treatment and ongoing medical care can improve the quality of life and prognosis for people with vasculitis.

What causes Vasculitis?

The causes of vasculitis are not fully understood by researchers. Most types of vasculitis are autoimmune diseases, that occur when the body’s natural defense system mistakenly attacks healthy tissues.

Researchers believe a combination of factors may trigger the inflammatory process, including infections, medications, genetic factors, environmental factors, allergic reactions, or another disease. However, the exact cause is usually unknown.

Who gets Vasculitis?

Vasculitis can affect people of all ages and races, although some forms may be more common among certain age or ethnic groups. Vasculitis usually, but not always, affects woman and men in equal numbers.

Types of Vasculitis?

There are many types of vasculitis, which are classified by the size and location of affected blood vessels. Your doctor will help determine the type of vasculitis you have and the most appropriate treatment.

MOST COMMON TYPES OF VASCULITIS (Classified by vessel size)

Large vessel

Aortitis
Giant cell arteritis
Polymyalgia rheumatic
Takayasu’s arteritis

Medium vessel

Kawasaki disease
Polyarteritis nodosa

Small vesel

Anti-GBM (Goodpasture’s) disease
Cryoglobulinemia
Cutaneous small-vessel vasculitis (formerly called
hypersensitivity)
IgA vasculitis (Henoch-Schonlein purpura)
Urticarial vasculitis (hypocomplementemic)

Small- and medium- sized vessel

Central nervous system angitis
Eosinophilic granulomatosis with polyangitis (EGPA/Churg-Strauss)
Granulomatosis with polyangitis (formerly Wegener’s)
Microscopic polyangiitis
Rheumatoid vasculitis

Arteries of various sizes

Behcet’s syndrome
Cogan’s syndrome

What are the symptoms of Vasculitis?

Vasculitis symptoms vary from patient to patient and defend on the type of vasculitis and affected tissues and organs. Symptoms may include:

Fatigue/weakness
Fever
Muscle and/or joint pain
Lack of appetite/weight loss
Rashes or skin lesions
Eye pain and redness/blurred vision
Chronic nasal, ear and/or sinus problems
Shortness of breath
Cough (or coughing up blood)
Abdominal pain
Severe headaches
Nerves problems, such as numbness, weakness, pain (neuropathy)
Blood or dark-colored urine, potentially indicating kidney problems
(Note: A patient can have kidney disease without having symptoms, so patients with vasculitis of any form should have regular urine tests.)

Complications

Serious vasculitis complications can occur, especially if the disease goes undiagnosed or untreated. Depending on the type of vasculitis and severity of conditions, complications can include organ damage or failure; blood clots; an aneurysm (an abnormal bulging of weakened blood vessel that can burst); heart problems; vision loss; and neuropathy, among others.

If you have the above symptoms, or others that you are concerned about, report them to your doctor as soon possible.

How is vasculitis diagnosed?

Diagnosing vasculitis can pose a challenge because the symptoms may be similar to those caused by other illnesses or diseases. Your doctor will take a detailed medical history and perform a physical exam. Depending on symptoms and the type of vasculitis suspected, your doctor may order laboratory tests such as urinalysis and blood tests; imaging studies such as X-rays, computed tomography (CT), or magnetic resonance imaging (MRI) scans; lung functions tests; or biopsy, when indicated.

A biopsy involves surgical removal of a small sample of affected organ or tissue, which is analyzed for signs of inflammation or tissue damage. A biopsy is usually obtained to confirm diagnosis, however this is not always feasible. In addition, a positive biopsy is not always a requirements to confirm the diagnosis before starting treatment.

How is vasculitis treated?

Treatment is based on numerous factors including the specific type of vasculitis, symptoms, organs affected, disease severity, lab results, age, overall health and more. It is essential to work closely with your doctor in developing a comprehensive treatment plan.

Treatment usually involves two phases: controlling the inflammation to achieve remission, and maintenance treatment to prevent relapse. Common treatments include the following:

Corticosteroids such as prednisone (or prednisolone) are often the first line treatment for vasculitis, to reduce inflammation.
For more serious forms of vasculitis, meditations that suppress the immune system are often prescribed, including methotrexate, azathioprine, mycophenolate mofetil, and cyclophosphamide.
Biologic agents such as rituximab, tocilizumab, and mepolizumab may be prescribed for specific types of vasculitis. Biologic medications are complex proteins derived originally from living organisms. They target certain parts of the immune system to control inflammation.
For very severe cases, other additional treatments include plasmapheresis (plasma exchange), intravenous gamma globulin, or surgery to restore blood flow.

Side effects

All the medications used to treat vasculitis have side effects. The including lowering your body’s ability to fight infection, potential bone loss (osteoporosis), and others. Your doctor may prescribe medictions to offset these side effects. Infections prevention is also very important. Talk to your doctor about getting vaccinated against influenza, pneumococcal (pneumonia) vaccination, and/or shingles vaccination, which can reduce your risk of infection.

Relapse

Even with effective treatment, relapses of vasculitis are common. Regular doctor visits and ongoing monitoring of lab and imaging tests are important in detecting relapses early.

Your medical team

Effective treatment of vasculitis often requires the coordinated efforts and ongoing care of a team of medical providers and specialist. In addition to your general practioner, patients may need to see: a rheumatologist (joints, muscles); clinical immunologist (immune system); nephrologist (kidneys), dermatologist (skin); respiratory physician (lungs); gastroenterologist (digestive system); ENT (ear, nose and throat) surgeon; cardiologist (heart); neurologist (brain/nervous system); or others as needed.

Living with Vasculitis

Coping with vasculitis can be overwhelming at times. Fatigue, pain, emotional stress, and medication side effects can take a toll on your sense of well-being, affecting relationships, work and other aspects of your daily life. Sharing your experience with family and friends, connecting with others through a support group, or talking with a mental health professional can help.

Outlook

There is no cure for vasculitis at this time, but with early diagnosis and proper treatment, many patients can lead full, productive lives. Outlook depends on a number of factors, including the form of vasculitis; affected organs; severity of disease; how soon it is diagnosed and treated; and whether there is an underlying condition, among others. Most forms of vasculitis are chronic, with periods of relapse and remission. In addition, medications used to treat vasculitis carry the risk of side effects, so follow-up medical care is essential.
Clinical studies are ongoing at multicenter research centers, including in the USA, the Vasculitis Clinical Research Consortium (VCRC), to better understand the risk factors and causes of vasculitis, investigate more effective and safer treatments, and work toward a cure. The Vasculitis Foundation encourages patients to consider participating in clinical research studies to help further understanding of vasculitis. Patients are also encouraged to join the Vasculitis Patient Powered Research Network (VPPRN), where they can provide valuable disease insight and information.

ANZVASC, the Australian and New Zealand Vasculitis Society, was formed in late 2018 to promote collaboration and excellence in vasculitis care and research in Australia and New Zealand. Patients and consumer are welcome to participate and can join as associate members.

For more information on vasculitis and vasculitis research, visit:
www.anzvasculitis.org
www.vasculitisfoundation.org

ANZVASC thanks the Vasculitis Foundation for permission to use this information, with minor changes for use in Australia and New Zealand