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Vasculitis

Overview

Blood vessel inflammation, called vasculitis, occurs when the blood vessels become inflamed. This inflammation can cause the blood vessel walls to get thicker, making the inside space smaller. This narrowing restricts blood flow, which can harm organs and tissues.

There are many different kinds of vasculitis, and most are uncommon. The inflammation can affect just one part of the body, or multiple areas. Some cases are short-lived, while others last a long time.

Anyone can develop vasculitis, although certain types are more common in specific age groups. In some cases, the condition might improve without treatment. However, most types of vasculitis need medicine to control the inflammation and prevent future problems. This medicine helps keep the inflammation under control and stops it from coming back (flares).

Symptoms

Vasculitis is a condition where the blood vessels become inflamed. Many types exist, but they often share similar symptoms.

Common signs and symptoms of vasculitis include:

  • General Symptoms: Feeling feverish, having headaches, feeling tired, losing weight, and experiencing general aches and pains are common.

  • Symptoms Related to Affected Body Parts: The specific symptoms depend on which part of the body the inflammation affects.

    • Digestive System: If the stomach or intestines are involved, you might feel pain after eating. This can sometimes lead to ulcers or holes (perforations) in the digestive tract, potentially causing blood in your stool.

    • Ears: Vasculitis can cause dizziness, ringing in the ears (tinnitus), and sudden hearing loss.

    • Eyes: Inflammation can make your eyes red, itchy, or burn. A serious type of vasculitis called giant cell arteritis can cause double vision, and temporary or permanent vision loss in one or both eyes. Sometimes, vision loss is the first sign of the illness.

    • Hands and Feet: Some types of vasculitis can lead to numbness or weakness in a hand or foot. The palms of your hands or soles of your feet might swell or become hard.

    • Lungs: If vasculitis affects the lungs, you might experience shortness of breath or cough up blood.

    • Skin: Red spots, lumps, or open sores on the skin can be signs of vasculitis. Sometimes, small bleeds under the skin appear as tiny red spots.

Important Note: If you notice any of these symptoms, it's crucial to see a doctor. Some types of vasculitis can get worse quickly, so early diagnosis is very important for getting effective treatment.

When to see a doctor

If you're experiencing any health concerns, schedule a visit with your doctor. Some forms of vasculitis can get worse quickly. Early detection is crucial for getting the right treatment. Don't delay if you have any worries; a prompt diagnosis is important for managing these conditions effectively.

Causes

Vasculitis, a condition where the blood vessels become inflamed, has a complicated cause. Scientists haven't pinpointed the exact reason for all cases. Sometimes, a person's genes play a role. In other cases, the body's immune system, which is supposed to fight off infections, mistakenly attacks the cells of the blood vessels.

Several things can trigger this mistaken attack. These include:

  • Infections: Some infections, like hepatitis B and hepatitis C, can sometimes confuse the immune system, leading it to attack blood vessels.
  • Cancer: Certain blood cancers can also cause the immune system to malfunction and attack blood vessels.
  • Immune system disorders: Diseases that affect the immune system, such as rheumatoid arthritis, lupus, and scleroderma, can sometimes lead to vasculitis. These diseases essentially make the immune system overactive, causing it to attack healthy tissues, including blood vessels.
  • Medications: Reactions to certain medicines can sometimes trigger the immune system to attack blood vessels. This is a less common cause, but it's important to remember that some drugs can have unexpected side effects.
Risk factors

Vasculitis can affect anyone, but certain factors might make some people more likely to develop it. Understanding these risk factors can help people take steps to protect their health.

Here are some things that can increase the chances of getting different types of vasculitis:

  • Age: Some types of vasculitis are more common in specific age groups. For example, giant cell arteritis is rarely seen before age 50, and Kawasaki disease typically affects children under 5.

  • Family history: If someone in your family has certain types of vasculitis, such as Behçet's disease, granulomatosis with polyangiitis, or Kawasaki disease, you might have a higher risk. This suggests a possible genetic link.

  • Lifestyle: Some lifestyle choices can increase the risk. Using cocaine can raise the chances of developing vasculitis. Smoking, especially if you're a man under 45, can significantly increase the risk of Buerger's disease, a type of vasculitis. It's important to note that smoking is a significant risk factor for many health problems.

  • Medications: Sometimes, certain medications can trigger vasculitis. Examples include hydralazine, allopurinol, minocycline, and propylthiouracil. If you're taking these medications, it's important to discuss any concerns with your doctor.

  • Infections: Having infections like hepatitis B or C might increase the risk of vasculitis. These infections can sometimes affect the body's immune system, potentially leading to vasculitis.

  • Immune disorders: People with conditions where their immune system mistakenly attacks their own body tissues (autoimmune disorders) are more likely to get vasculitis. Examples include lupus, rheumatoid arthritis, and scleroderma. These conditions can disrupt the body's normal processes, sometimes leading to vasculitis.

  • Gender: Some types of vasculitis are more common in certain genders. For example, giant cell arteritis is more common in women, while Buerger's disease is more common in men. This difference in risk may be due to biological factors or other influencing elements.

It's important to remember that these are just possible risk factors. Not everyone with these factors will develop vasculitis, and many people without these factors can still get it. If you're concerned about your risk, talking to your doctor is always the best approach.

Complications

Vasculitis problems depend on how severe the condition is and what type it is. Sometimes, side effects from the medicines used to treat vasculitis can also cause issues. Here are some potential complications:

  • Damage to organs: Some forms of vasculitis are serious and can harm important organs in your body. This damage can affect how well those organs work.
  • Blood clots and bulges in blood vessels: Vasculitis can sometimes cause blood clots to form, blocking blood flow to parts of the body. Less commonly, it can cause a blood vessel to weaken and bulge, forming a bulge called an aneurysm.
  • Vision loss: A type of vasculitis called giant cell arteritis can lead to vision problems, or even blindness, if not treated.
  • Increased risk of infections: Medicines used to treat vasculitis can sometimes weaken the body's natural defenses against illness. This makes people with vasculitis more susceptible to infections.

Andy Grundstad, a 35-year-old agronomist from North Dakota, was very sick for a long time. "I felt like I was 80 years old," he said. Andy had been diagnosed with vasculitis as a child, and throughout his life, he struggled with various health problems. Doctors had difficulty making a precise diagnosis and finding effective treatment. Things got worse for Andy after a stroke in 2017. High fevers and joint pain became nearly daily occurrences, and a second stroke happened in 2020.

Diagnosis

Your doctor will likely start by asking about your health history and doing a physical exam. They might also order one or more tests to either rule out other conditions that could be mistaken for vasculitis, or to confirm a vasculitis diagnosis. These tests can include:

Blood Tests: These check for signs of inflammation. For example, a high level of C-reactive protein (CRP) suggests inflammation. A complete blood count (CBC) checks if your red blood cell levels are normal. Some blood tests look for specific antibodies, like the anti-neutrophil cytoplasmic antibody (ANCA) test. These antibodies can help pinpoint vasculitis.

Imaging Tests: These non-invasive tests help doctors see which blood vessels and organs might be affected by vasculitis. They can also be used to see if treatment is working. Common imaging tests for vasculitis include:

  • X-rays: These use radiation to create images of bones and some soft tissues.
  • Ultrasound: This uses sound waves to create images of structures inside the body.
  • CT scans (Computed Tomography): These use X-rays and computers to create detailed images of the body's internal structures.
  • MRI scans (Magnetic Resonance Imaging): These use magnetic fields and radio waves to create detailed images of the body's soft tissues.
  • PET scans (Positron Emission Tomography): These use radioactive substances to create images of the body's activity, which can help identify areas of inflammation.

Angiography: This test takes detailed images of your blood vessels. A thin tube (catheter) is inserted into a large artery or vein. A special dye is injected through the catheter, allowing X-rays to show the blood vessels clearly.

Biopsy: Sometimes, a small piece of tissue from the affected area is removed surgically (biopsy). Doctors examine this tissue under a microscope to see if there are signs of vasculitis. This helps confirm the diagnosis.

Treatment

Vasculitis treatment aims to reduce inflammation and address any underlying causes.

A common medicine for controlling vasculitis inflammation is a corticosteroid, like prednisone.

Long-term corticosteroid use can cause serious side effects, including weight gain, diabetes, and weakened bones. Doctors try to prescribe the lowest possible dose if the treatment needs to be long-term.

Other medicines are often used alongside corticosteroids to help reduce inflammation and allow the corticosteroid dose to be lowered more quickly. The best choice depends on the specific type of vasculitis. These additional medications might include methotrexate, azathioprine, mycophenolate, cyclophosphamide, tocilizumab, or rituximab.

The exact medicines used depend on the type and severity of vasculitis, which organs are affected, and any other health issues you have.

Living with vasculitis can be challenging due to medicine side effects. Here are some helpful tips:

  • Learn about your condition: Understanding vasculitis and its treatment, including potential side effects, is key. Tell your doctor about any changes in your health.

  • Get vaccinated: Staying up-to-date on vaccinations (like flu and pneumonia shots) can help prevent infections, which can be more likely with certain medications. Talk to your doctor about your vaccination needs.

  • Build a support network: Family, friends, and even support groups can be invaluable. Ask your doctor or nurse about local support groups for people with vasculitis.

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Disclaimer: August is a health information platform and its responses don't constitute medical advise. Always consult with a licenced medical professional near you before making any changes.

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