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Autoimmune Vasculitis - Causes, Symptoms, and Treatment.

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Autoimmune vasculitis is an inflammation of the blood vessels related to autoimmunity. The body’s immune system is attacked by the blood vessels by mistake.

Written by

Dr. Narmatha. A

Medically reviewed by

Dr. Kaushal Bhavsar

Published At April 18, 2023
Reviewed AtApril 18, 2023

What Is Autoimmune Vasculitis?

Autoimmune vasculitis is an autoimmune-related inflammation of the blood vessels. It occurs when the host’s immune system attacks the blood vessels by mistake. It can occur due to an infection, a drug, or a disease. The etiology is often unknown.

Autoimmune vasculitis can affect arteries, veins, and blood capillaries. Arteries carry pure blood from the heart to the body’s organs. Veins carry impure blood back to the heart. Capillaries are small blood vessels that connect the small arteries and veins. When there is inflammation of the blood vessel, it usually becomes narrow, making it more difficult for blood to pass through.

The artery closes off completely so that the blood cannot pass through. Sometimes the artery stretches and weakens itself so much that it starts to bulge. This bulge is called an aneurysm. Dangerous bleeding can be caused inside the body if the artery bursts. If blood flow is restricted, it can damage organs and tissues. Symptoms of vasculitis usually include high fever, edema, and a general sense of feeling ill. It is not a single disease entity but rather a group of rare diseases. These diseases include Kawasaki disease, granulomatosis with polyangiitis, Behcet’s disease, Goodpasture’s disease, and others.

What Are the Causes of Autoimmune Vasculitis?

The exact etiology of autoimmune vasculitis is not completely understood. Hereditary factors might play a role in the pathogenesis of autoimmune vasculitis. Generally, the host's immune system is attacked by the blood vessel cells by mistake. Possible trigger agents for this autoimmune reaction include:

  • Viral infections, such as hepatitis B and hepatitis C.

  • Blood cancers (leukemias, multiple myelomas).

  • Autoimmune system diseases, such as rheumatoid arthritis, lupus erythematosus, and scleroderma.

  • Certain drug reactions.

What Are the Risk Factors of Autoimmune Vasculitis?

Vasculitis can happen to anyone. Factors that may increase the risk of certain disorders include:

  • Age- Giant cell arteritis rarely occurs before fifty years of age, while Kawasaki disease most commonly occurs in children younger than five years old.

  • Family Medical History- Behcet's disease, granulomatosis with polyangiitis, and Kawasaki disease have a familial history.

  • Lifestyle Changes- Cocaine usage can increase the risk of developing autoimmune vasculitis. Tobacco smoking can increase the risk of Buerger's disease.

  • Certain Drugs- Autoimmune vasculitis can be triggered by certain medications such as Hydralazine, Allopurinol, Minocycline, and Propylthiouracil.

  • Viral Infections- Hepatitis B or hepatitis C viral infections can increase the risk of autoimmune vasculitis.

  • Autoimmune Disorders- People suffering from autoimmune disorders (in which the immune system of the host is mistakenly attacked by their own antibodies) are at a higher risk of developing autoimmune vasculitis. Examples include systemic lupus erythematosus, rheumatoid arthritis, and scleroderma.

  • Sex- Giant cell arteritis is much more prevalent in women, while Buerger's disease is more commonly found in men.

What Are the Symptoms of Autoimmune Vasculitis?

General signs and symptoms of autoimmune vasculitis include:

  • High fever.

  • Severe headache.

  • Tiredness.

  • Unintentional weight loss.

  • Muscle and joint pain.

  • Body ache.

Other signs and symptoms are usually related to other parts of the body that are affected, including:

  • Digestive System- If the stomach and the intestines get affected, the patient might experience pain after having food. Stomach ulcers and intestinal perforations may occur that may result in blood in the stool.

  • Ears- Confusion, dizziness, ringing sounds in the ears, and sudden hearing loss may occur.

  • Eyes- Autoimmune vasculitis can cause redness, burning, and itching to the eyes Giant cell arteritis can lead to double vision and temporary or permanent loss of vision in one or both eyes. This is usually the first sign of the disorder.

  • Upper and Lower Extremities- Numbness or weakness in either hands or feet can be caused due to autoimmune vasculitis. There can be swelling seen on the hands and feet.

  • Lungs- There can be difficulty breathing or even coughing blood if the autoimmune vasculitis affects the patient's lungs.

  • Skin- Bleeding underneath the skin can appear as red spots. Autoimmune vasculitis can cause bumps or open sores on the skin.

What Are the Diagnostic Tests for Autoimmune Vasculitis?

The diagnosis of the autoimmune condition requires the following:

  • Thorough Family Medical History - The healthcare professional should thoroughly examine the patient to rule out another medical disease with similar clinical symptoms.

  • Blood Tests - These tests are performed to check the signs of inflammation, such as increased erythrocyte sedimentation rate, increased C-reactive protein, increased white blood cells, anemia, and increased eosinophil count.

  • X-Rays - They are used to examine where the artery is damaged and the amount of destruction that is done.

  • Magnetic Resonance Imaging - It isa test that uses electromagnetic energy waves to produce images of the pathology on the computer.

  • Computed Tomography - It is also used to view the images of internal organs like the blood vessels.

  • Angiography - This is the procedure to examine the X-ray images of the internal part of the blood vessels. A long, slender catheter tube is inserted into a large artery in the arm or the groin region by the doctor and attached to the artery. The catheter is then used to inject the contrast dye into the blood vessel to take the X-ray images. This contrasting material helps to differentiate the pathological blood vessel from other blood vessels in the X-ray images.

  • Positron Emission Tomography - A radioactive substance is injected through a needle into the arm of the patient. This substance is detected by a scanner while traveling through the body to form the images.

  • Ultrasound - Here, the sound waves are used to produce images of the blood vessels.

  • Biopsy - It will describe the pattern of blood vessel inflammation.

  • Serologic Tests - It is an antibody test used to screen the antibodies produced by the body that attack its own body cells in autoimmune disorders.

What Is the Treatment of Autoimmune Vasculitis?

Treatment is mainly aimed at reducing the signs of inflammation of the blood vessels and suppressing the immune system. Corticosteroids are mainly the drugs of choice. Immunosuppressant drugs are also considered to modulate the host’s immunity. In case of infections, antibiotics may also be prescribed. The particular organs involved may require specific medical treatment.

When to Visit a Doctor?

Take an appointment with your healthcare professional if you have any signs or symptoms that are worrying you. Autoimmune vasculitis can worsen quickly and rapidly, so early diagnosis is the key to achieving effective treatment.

Conclusion

Autoimmune vasculitis is not a single but a rare group of autoimmune disorders involving vasculitis. Inflammation of the blood vessels occurs due to an autoimmune reaction. Due to the rarity of these diseases, the research has been challenging. Therefore, efforts should be made to encourage researchers to do new studies in this field to seek better treatment and therapeutics.

Frequently Asked Questions

1.

How Long Does Vasculitis Therapy Typically Last?

Depending on the nature and severity of the vasculitis, vasculitis treatment can take anywhere from a few months to a few years. To control the illness and avoid relapses, it may occasionally be essential to get long-term or even permanent therapy. Treatment plans are made to meet the needs of every patient.

2.

What Might Be the Origin or Initiating Factors of Autoimmune Inflammatory Vasculitis?

Although the precise cause of autoimmune inflammatory vasculitis is unknown, it is thought to result from genetic predisposition and environmental factors. Blood vessel inflammation may result from an immunological reaction brought on by infections, medicines, and exposure to certain chemicals.

3.

Is There a Permanent Remedy for Autoimmune Vasculitis, or Can It Be Controlled With Medication?

Although there is often no permanent treatment for autoimmune vasculitis, it is frequently adequately controlled by medicines. Corticosteroids and immunosuppressive medications are frequently used to reduce inflammation and stop flare-ups. The goal of treatment is to bring about remission and keep it going so that patients may continue to manage their medications and have relatively normal lives.

4.

What Measures Can Be Taken to Prevent the Progression of Vasculitis?

Medical therapy is generally required to stop the progression of vasculitis, an inflammation of blood vessels. Doctors frequently give immunosuppressive treatments such as corticosteroids or immunomodulatory therapies to treat the underlying immune system failure. It is essential to schedule routine follow-up visits to keep track of disease activity and modify treatment as necessary. In addition, lifestyle changes like quitting smoking and eating a balanced diet can promote general health and lessen inflammation. To stop vasculitis from worsening and leading to more serious problems, early detection and immediate treatment are essential.

5.

What Self-Care Techniques Are Suggested for People With Vasculitis?

Self-care for people with vasculitis should emphasize symptom management and promoting general well-being. This includes following the instructions on prescription drugs, going to routine doctor's appointments, eating a nutritious diet, exercising regularly, and managing stress through therapy or relaxation techniques. It also includes reaching out for social support. In addition, cutting back on alcohol and cigarette use can help vasculitis patients live better lives.

6.

What Safety Measures Should People With Vasculitis Take To Prevent Aggravating Their Disease or Causing Flare-Ups?

Safety precautions should come first for those with vasculitis to avoid illness flare-ups and deterioration. This entails taking prescribed medications as directed, adhering to a physician's recommended course of treatment, avoiding triggers like specific drugs or infections, managing strain, leading a healthy lifestyle, and promptly seeking medical attention for any worrisome symptoms or changes in their condition. Additionally, patients need to follow any additional precautions advised by their healthcare professional and be knowledgeable about their particular kind of vasculitis if they want to manage the condition while lowering risks properly.

7.

What Is the Extent of Autoimmune Vasculitis and What Are the Possible Side Effects?

The body's organs and tissues may be impacted by autoimmune vasculitis, which can cause inflammation and damage. Depending on the organs involved, possible side effects might vary from skin rashes and joint discomfort to more serious issues, including organ damage, neurological issues, and even possibly fatal results.

8.

Are There Known Triggers or Factors That Can Contribute to the Development or Exacerbation of Autoimmune Vasculitis?

Infections, certain drugs, chemicals in the environment, genetics, and autoimmune disorders can all cause or worsen autoimmune vasculitis. Sometimes, infections and drug interactions can start or exacerbate vasculitis episodes.

9.

What Is the Life Expectancy of Individuals Diagnosed With Vasculitis?

Vasculitis patients' life expectancy varies greatly based on the kind and severity of their condition, the efficacy of their therapy, and the occurrence of comorbidities. Many people can live reasonably normal lives with the right medical treatment, but severe instances can be fatal. Sticking to treatment regimens and having routine medical checkups is essential for better results.

10.

Is It Advised to Relax or Adopt Certain Lifestyle Changes as Part of Managing Vasculitis?

To manage vasculitis, various lifestyle adjustments are indicated. To improve general well-being and illness management, this may involve lowering stress, keeping a balanced diet, engaging in physical activity within one's capabilities, abstaining from cigarettes and excessive alcohol, and obtaining enough rest.

11.

What Recent Developments Have Been Made in the Management of Vasculitis?

For adult patients with severe active anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis, the Food and Drug Administration has authorized Avacopan as an add-on medication to conventional therapy, which includes glucocorticoids.

12.

Can Individuals With Vasculitis Achieve and Maintain a Healthy Lifestyle?

Vasculitis sufferers can lead healthy lifestyles by following their treatment regimens, controlling their stress levels according to dietary advice, engaging in moderate physical activity, and abstaining from smoking and binge drinking. It's crucial to have regular medical surveillance.

13.

What Function Does Immunotherapy Serve in the Management of Vasculitis?

To control vasculitis, immunotherapy modifies the immune system. In many instances, it aids in controlling the progression of the disease and lessens symptoms by working to decrease the immunological response that causes inflammation and blood vessel damage.

14.

Are Surgical Interventions Ever Necessary in the Management of Vasculitis?

In order to control vasculitis, immunotherapy modifies the immune system. In many instances, it aids in controlling the progression of the disease and lessening symptoms by working to decrease the immunological response that causes inflammation and blood vessel damage.

15.

Are There Any Dietary Suggestions or Particular Items That May Aid in the Management or Prevention of Vasculitis?

Although there is no particular diet that may prevent vasculitis, eating a balanced diet high in fruits, vegetables, whole grains, and lean meats can help promote general health. Managing the related high blood pressure may be aided by reducing salt consumption. For individualized nutritional advice, speaking with a doctor or dietician is advised.

16.

What Effects Does Vasculitis Have on a Person’s Day-To-Day Functioning and General Well-Being?

Vasculitis may substantially negatively influence a person's ability to function and overall health. Fatigue, discomfort, and organ involvement are among the symptoms that can impair physical and emotional capacities, impacting relationships, jobs, and general quality of life. To lessen these impacts, appropriate management and assistance are crucial.

Dr. Kaushal Bhavsar
Dr. Kaushal Bhavsar

Pulmonology (Asthma Doctors)

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