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General Vasculitis

About Vasculitis

Last Updated on February 5, 2024

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 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 aggressive treatment and 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.

The cause of vasculitis is not fully understood. Vasculitis is classified as an autoimmune disorder, which occurs 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, genetics, the environment, allergic reactions or another disease. However, the exact cause is usually unknown.

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

Vasculitis symptoms vary from patient to patient and depend 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 (including coughing up blood)
  • Abdominal pain
  • Severe headaches
  • Nerve problems (numbness, weakness, pain)
  • Bloody or dark-colored urine, potentially indicating kidney problems (Note: A patient can have kidney disease without having symptoms; therefore, patients with vasculitis should have urine tests.)

Serious vasculitis complications can occur, especially if the disease goes undiagnosed or untreated. Depending on the type of vasculitis and severity of condition, complications can include organ damage or failure, blood clots, an aneurysm (an abnormal bulging of a weakened blood vessel that can burst), heart problems, vision loss, neuropathy, and lung bleeding, among others. If you have the above symptoms, or others that you are concerned about, report them to your doctor as soon as possible.

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 your 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, or lung function tests. A biopsy of the affected tissue or organ is usually obtained to confirm diagnosis but is not always feasible. Biopsy is not always required to confirm the diagnosis before starting treatment, which shouldn’t be delayed.

Treatment is based on numerous factors including the specific type of vasculitis, symptoms, organs affected, disease severity, laboratory test 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 relapses. Common treatments include the following:

  • Glucocorticoids such as prednisone are often the first line of treatment for vasculitis to reduce inflammation. Glucocorticoids are also immunosuppressive medications.
  • For more serious forms of vasculitis, other medications 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 from living organisms; they target certain parts of the immune system to control inflammation.
  • For very severe cases, other additional treatments may include plasmapheresis (plasma exchange), intravenous gamma globulin, or surgery to restore blood flow.

Learn More About Treatments for Vasculitis

In 2021 the American College of Rheumatology (ACR) published guidelines for the management of certain vasculitides, that were also endorsed by the Vasculitis Foundation (VF). Clinical practice guidelines are developed to reduce inappropriate care, minimize geographic variations in practice patterns, and enable effective use of health care resources. Guidelines and recommendations developed and/or endorsed by the ACR are intended to provide guidance for particular patterns of practice and not to dictate the care of a particular patient. The application of these guidelines should be made by the physician in light of each patient’s individual circumstances. Guidelines and recommendations are subject to periodic revision as warranted by the evolution of medical knowledge, technology, and practice.

All the medications used to treat vasculitis have side effects. These include lowering your body’s ability to fight infection, potential bone loss (osteoporosis), and others. Your doctor may prescribe medications to offset these side effects. Infection prevention is also very important. Talk to your doctor about getting vaccines (e.g., flu shot, pneumonia and/or shingles vaccination), which can reduce your risk of infection.

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

Effective treatment of vasculitis often requires the coordinated efforts and ongoing care of a team of medical providers and specialists. In addition to a primary care provider, patients may need to see a:

  • Rheumatologist (joints, muscles, immune system)
  • Dermatologist (skin)
  • Pulmonologist (lungs)
  • Gastroenterologist (digestive system)
  • Otolaryngologist (ear, nose, and throat)
  • Immunologist (immune system)
  • Nephrologist (kidneys)
  • Cardiologist (heart)
  • Neurologist (brain/nervous system) or others as needed

Learn More About Building Your Treatment Team

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.

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 several 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.

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