Vasculitis

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What is vasculitis?

Vasculitis is an inflammation of the blood vessels, causing the walls of these vessels to thicken, circulatory pathways to narrow, and blood vessel integrity to collapse. This less common, yet serious, condition restricts blood flow, which can cause organ or tissue damage to the lungs, nerves or skin. There are many types of vasculitis that range in severity and symptoms, depending on the area of the body and type of blood vessel it affects. Vasculitis is also known as angiitis or arteritis. It is often caused by an underlying autoimmune disease.

Vasculitis affects people in widely differing ways. Some people have mild vasculitis; some have one episode and then it goes away and never comes back; others have chronic vasculitis. Severe vasculitis can lead to disability and is fatal is some cases.

The disease can affect both men and women of all ages. However, there are some types of vasculitis that affect specific population groups. Kawasaki disease is a type of vasculitis seen most often in children under 5, while giant cell arteritis affects only people older than 50.

Some forms of vasculitis have no outward symptoms, but without treatment, ongoing blood vessel inflammation damages your vital organs. If you have risk factors for the condition, such as a family history of vasculitis, or any symptoms that concern you, visit your doctor. Blood tests, imaging, and tissue biopsies can help determine if you have vasculitis.

What are the symptoms of vasculitis?

The signs and symptoms of vasculitis depend on which type you have and the organs and body tissues it affects. The types are generally categorized by the size of the blood vessels involved—from tiny capillaries to large arteries.

Inflammation, decreased blood flow, and tissue damage produce vasculitis symptoms. You may experience symptoms suddenly or gradually.

Common vasculitis symptoms include:

  • Cough

  • Fatigue

  • Fever

  • General aches and pains

  • Loss of pulse in a limb

  • Night sweats

  • Numbness or weakness in an extremity (hand or foot)

  • Shortness of breath

  • Skin disruptions including lumps (nodules) on the skin, red spots or rash (purpura), or sores (skin ulcers)

  • Weight loss

Some of the more common types of vasculitis carry specific symptoms. To diagnose a specific type of vasculitis, doctors consider a patient’s signs and symptoms, test results, and medical history. Here are symptoms associated with some of these conditions, grouped by the type of blood vessel that is most often involved:

Large arteries: the aorta and major branches

  • Cogan’s syndrome affects your eyes and ears; symptoms include eye inflammation, sudden deafness or other hearing problems, and vertigo

  • Giant cell arteritis affects arteries in your head, such as your temples; symptoms include sudden vision loss or such problems as blurred or double vision, headaches, scalp tenderness, and jaw pain

  • Polymyalgia rheumatica most often affects the shoulders; symptoms include stiffness and pain in the muscles of the neck, shoulders, lower back, hips, and thighs

  • Takayasu’s arteritis affects large arteries such as the aorta; symptoms include tiredness, feeling unwell, fever, night sweats, sore joints, and weight loss

Medium-sized arteries

  • Kawasaki disease affects coronary arteries, typically in children under 5; symptoms include redness in eyes, mouth and skin; enlarged lymph nodes, fever, rash, eye inflammation

  • Polyarteritis nodosa affects multiple systems including nerves, skin, kidneys, and heart; symptoms include fever, feeling of being unwell, weight loss, muscle and joint aches, rash, bumps under the skin, and stomach pain

Small and/or medium arteries

  • Eosinophilic granulomatosis with polyangiitis (Chaug-Strauss syndrome) affects the kidneys, lungs and nerves in your legs and arms; symptoms include asthma, nerve pain and abnormal lumps known as granulomas

  • Granulomatosis with polyangiitis (also called Wegener's granulomatosis) affects your nose, sinuses, throat, lungs and kidneys; symptoms include stuffy nose, nosebleeds, sinus infections, and granulomas

  • Hypersensitivity vasculitis, or cutaneous vasculitis affects your skin, most often on your lower legs; symptoms include red spots on the skin

Small arteries

  • IgA (Immunoglobulin A) vasculitis (Henoch-Schönlein) most often affects the skin, gastrointestinal tract, and kidneys; symptoms include a characteristic reddish-purple vasculitic rash, abdominal pain, swollen and painful joints, and blood in the urine

  • Microscopic polyangiitis affects small blood vessels in the kidneys, lungs, and other areas; symptoms include fever, weight loss, muscle aches, coughing up blood (if lungs are affected), and rash

Any size artery or vein

  • Behcet’s disease affects the mouth, eyes and genitals; symptoms include painful mouth and genital sores, acne-like skin lesions, and eye pain and inflammation

  • Relapsing polychondritis affects cartilage, such as in the nose or ears, and other connective tissue throughout the body; symptoms include redness or pain in the eyes, hoarseness, cough, difficulty breathing, rash, and breastbone pain

What causes vasculitis?

Vasculitis causes include a combination of genetic and environmental factors. The cause may vary by type of vasculitis. Some types are believed to be related to defective genes you inherit. When this occurs or when no other cause is known, a doctor makes a diagnosis of primary vasculitis.

Vasculitis can be triggered by an autoimmune response, which occurs when your immune system goes awry and attacks an element within your body. In the case of vasculitis, your immune system mistakenly attacks your blood vessels. This autoimmune response may be sparked by prior infections, current medical conditions, or medication reactions. These types of vasculitis are called secondary, because the vasculitis is part of another primary medical condition.

What are the risk factors for vasculitis?

Risk factors vary depending on whether vasculitis is secondary or primary. Risk factors for primary vasculitis include a family history of vasculitis, however, not all people with risk factors will develop vasculitis.

Risk factors for secondary vasculitis include:

How is vasculitis treated?

Vasculitis treatment varies depending on the type and how severe it is. If you have secondary vasculitis, your doctor may be able to treat the underlying cause.  

Medications for vasculitis

Medication is the most common treatment to help alleviate symptoms of vasculitis or to prevent it from worsening. Common types of medication include:

  • Corticosteroids, such as prednisone. These can reduce inflammation but can have long-term serious side effects, such as diabetes, high blood pressure, glaucoma, osteoporosis or greater susceptibility to infection

  • Immune suppressants including cyclophosphamide or methotrexate (for milder cases). These have less serious side effects than steroids, but can put patients at risk of infection.

  • Newer drugs that treat autoimmune and inflammatory diseases such as rituximab

  • IV immunoglobulin (IVIg), which may be given to people with severe vasculitis

Surgery for vasculitis

Surgery is not a common treatment for vasculitis, but possible treatments for vasculitis-related problems include:

  • Bypass grafting to redirect blood flow around blocked vessels

  • Sinus surgery

  • Kidney transplant

What are the complications of vasculitis?

Some people may never experience complications of vasculitis. Some patients may suffer from arthritis, nose or sinus problems. More severe complications include:

  • Organ damage including to lungs, heart, kidneys or brain

  • Blood clots and aneurysms

  • Vision loss or blindness

 Medication-related complications of vasculitis

Some of the medicines doctors prescribe for vasculitis can cause complications too. Patients on immunosuppressant therapy are more vulnerable to:

  • Infections including such life-threatening conditions as pneumonia or blood infection (septicemia) leading to sepsis

  • Infertility

  • Increased risk of cancer

  • Reactivated hepatitis B (for patients on rituximab)

Reducing your risk of complications from vasculitis

Work with your doctor to help reduce the chance of complications. See your doctor regularly and report any troublesome symptoms. You may be able to keep vasculitis from becoming worse or delay its progression by:

  • Following your doctor’s treatment plan including going to all follow-up visits

  • Having vascular surgery if necessary to repair or treat such vascular conditions as aneurysms and blood clots

  • Maintaining a healthy diet

  • Taking medicine your doctor prescribes

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Medical Reviewer: William C. Lloyd III, MD, FACS
Last Review Date: 2020 Jan 24
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