What Is Vasculitis?

Overview

Inflammation is the normal response of the immune system to injury or infection. The immune system sends the inflammatory cells, and they attack the bacteria or heal the damaged tissue. The inflammation may cause pain, swelling, heat and raised skin at the area of injury.

Vasculitis is a rare autoimmune condition that causes inflammation in the blood vessels. The swelling in the vessels causes narrowing, which makes the blood flow difficult, and this damages the tissue and organs. Arteries, veins, and capillaries are the types of blood vessels that carry blood throughout the body.

Autoimmune diseases occur when the immune system abnormally reacts and attacks the healthy normal cells and tissues of the body, which leads to swelling and narrowing. Over time, the inflammation can cause organ damage and serious complications.

Types of vasculitis

There are different types of vasculitis, and they are divided according to the blood vessels they affect.

• Large Vessel Vasculitis
  • Polymyalgia rheumatic: a type of vasculitis where muscle stiffness is the main symptom, and there is pain and stiffness in the shoulder, neck and hip. This vasculitis can lead to extreme tiredness, fever, depression and weight loss
  • Takayasu’s arteritis: This is a rare type of vasculitis that affects the main artery (aorta) from the heart. This carries blood from the heart to the rest of the body. The inflammation causes narrowed or blocked arteries, which can lead to weakening or bulge (aneurysm) and rupture of the blood vessels. This causes fever, extreme tiredness, weight loss, muscle pain, joint pain, chest pain, and high blood pressure, which results in heart failure or stroke.
  • Temporal arteritis: Temporal arteritis (Giant cell arteries) is inflammation that occurs in the lining of the arteries or side of the artery head. It commonly affects the arteries in the temples causing severe frequent headaches (pain in temples), and pain in the jaw while eating and talking, it affects the vision and causes double vision or loss of vision.¹

• Medium Vessel Vasculitis
  • Buerger’s disease: Buerger’s disease is a condition affecting the small and medium veins and arteries which restricts the blood flow to the hands and feet. Clots are developed inside the blood vessels, which leads to skin ulcers and gangrene (death of body tissue) formation in the fingers and toes. When the nerves are affected, there will be numbness and tingling sensations in the fingers and toes.
  • Cutaneous vasculitis: Cutaneous vasculitis is the inflammation affecting the arteries or veins or maybe both small or medium-sized blood vessels in the skin. The whole body is covered with skin, so it can affect any part of the body. The most common symptoms appear as small marks on the skin which is flat and raised, or look like bruises which do not fade away.²
  • Kawasaki disease: Kawasaki disease is commonly seen in children. It affects all the arteries, and also the coronary artery, which supplies the blood to the heart. When the arteries are inflamed, they become weak and stretched, and this leads to the narrowing and sometimes to the tearing of the blood vessels. When the blood supply is limited, it can cause heart disease. 
  • Polyarteritis nodosa: Polyarteritis nodosa is a condition where the small and medium-sized arteries are inflamed. This condition affects the skin and joints: peripheral nerves, gastrointestinal tract, heart, eyes, and kidneys. The symptoms occur according to the organ affected and are accompanied by common symptoms like fever, fatigue, loss of appetite, rashes, numbness and pain.¹

• Small Vessel Vasculitis
  • Behcet’s syndrome: Behcet’s syndrome is a rare disease which can cause inflammation of the blood vessels throughout the body. Common symptoms are mouth sore or ulcers, eye inflammation, skin rashes, skin lesions and genital sores. Treatment is given to reduce the signs and symptoms of the disease and to prevent the disease from leading to serious complications like blindness.
  • Churg-Strauss syndrome: Churg-strauss syndrome is a rare inflammation of the blood vessels, which restricts the blood flow to the organs and the tissues leading to permanent damage. It is also known as eosinophilic granulomatosis with polyangiitis (EGPA). Symptoms vary from person to person, where in some people it mild symptoms can appear and in others may be life-threatening complications. The most common symptoms are asthma, chronic sinusitis, increased white blood cells (Eosinophils), gastrointestinal bleeding, pain and numbness in the hands and feet.
  • Henoch-schonlein purpura: Henoch-schonlein purpura is the inflammation of the small blood vessels, which can bleed into the skin, causing a reddish-purple rash-like appearance. It is commonly seen in the lower legs and buttocks. This condition can affect the skin, joints, intestines and kidneys.⁴
  • Microscopic polyangiitis: Microscopic polyangiitis is a rare inflammation of the blood vessels which can lead to organ damage. The inflammation can cause weakened or stretched blood vessels, leading to aneurysms. It mainly affects the kidneys, lungs, nerves, skin and joints.
  • Granulomatosis with polyangiitis: It is a rare disease affecting the blood vessels, mainly the respiratory tract and kidneys. It is also known as Wegener’s granulomatosis. The inflammation causes bulges in the blood vessels and narrowed blood vessels and can cause inflammatory tissue masses known as granulomas. This can lead to reduced amounts of blood and oxygen reaching other body tissues and organs.
  • Cryoglobulinemia: Cryoglobulinemia is a condition where the cryoglobulins, which are the abnormal proteins in the blood, start to clump together. This can lead to decreased blood circulation and can also affect the skin, joints, nerves and especially the kidneys and liver.³

Causes and risk factors

Vasculitis is an autoimmune disease. Autoimmune disease means when the immune system attacks the healthy cells in the body instead of protecting them; the cause for this is unknown. Factors that may increase the chances of getting vasculitis are: 

1. Age: Most commonly, they occur before the age of 50. Kawasaki disease is commonly seen in children below the age of 5 years.⁵
2. Sex: Vasculitis can affect any sex, but temporal vasculitis is commonly seen in people assigned female at birth (PAFB), and Buerger’s vasculitis is commonly seen in people assigned male at birth (PAMB).
3. Family history: Some people are more prone to have the disease in cases where their family members have certain types of vasculitis like Behcet’s disease, granulomatosis with polyangiitis and Kawasaki disease.
4. Lifestyle choices: Individuals who have the habit of using cocaine and smoking tobacco have a higher risk of developing vasculitis.
5. Medications: Usage of certain medications for certain diseases for a very long time can develop a risk of getting vasculitis; some medicines are hydralazine, allopurinol, minocycline and propylthiouracil.
6. Diseases: Certain diseases can put a person at high risk for vasculitis, like hepatitis B and C; there are immune disorder diseases like rheumatoid arthritis, lupus and scleroderma.

Signs and symptoms

Common symptoms of vasculitis are: 

• Fever
• Headache
• Rashes, redness or slightly elevated skin.
• Fatigue
• Difficulty in breathing
• Weight loss
• Joint pain
• Numbness in hands and feet.
• Kidney problems
• Stroke

Diagnosis

The healthcare provider may take the patient's medical history and perform a physical examination. A rheumatologist is a doctor who treats inflammatory diseases. Other diagnostic techniques are: 

1. Blood test: Blood tests can help diagnose vasculitis by testing c-reactive proteins and antibodies.
2. Imaging tests: X-ray, MRI, ultrasound, computed tomography (CT) and positron emission tomography (PET) are non-invasive imaging techniques that help in finding the blood vessel affected and also help in knowing how much it is being affected.
3. Angiogram: It is a diagnostic procedure that uses imaging techniques to know about the blood vessels. In this procedure, a catheter is placed, and a dye is injected into the artery and vein, which flows through the artery and vein and helps find the block.
4. Biopsy: A biopsy is a procedure where the doctor takes a sample of the affected area and then examines the tissues.

Treatment and management

The main way to treat this condition is to manage the symptoms and reduce the severity.

Vasculitis surgery: The doctor suggests surgery according to the damage to the blood vessels and the organs affected.

Medicines: Corticosteroid drugs are prescribed to reduce the inflammation associated with vasculitis. Immunosuppressants are given to prevent the immune system from attacking healthy cells instead of protecting them.

Prognosis

Vasculitis cannot be cured, but symptoms can be reduced and, with proper treatment, can prevent tissue and organ damage. Regular monitoring and health checkups will be needed for life long.

Prevention

• High-risk patients should be monitored regularly 
• Should take proper treatment for their disease condition. 
• Following a healthy diet. 
• Doing regular exercise.
• Avoiding the usage of tobacco and cocaine

FAQs

Is vasculitis a serious disease?

Vasculitis is a minor problem if it affects the skin, but it becomes more serious when it affects the organs like the heart and kidneys. 

What is the cause of vasculitis?

Vasculitis is an autoimmune disorder where the exact cause is unknown. However, certain health conditions and habits can put the person at high risk of getting vasculitis.

When should I consult a doctor?

When new symptoms occur and the old symptoms of the disease get worse, it is best to consult the doctor as soon as possible. 

Summary

Vasculitis is a rare autoimmune condition that causes inflammation in the blood vessels. The swelling in the vessels causes narrowing, which makes the blood flow difficult, and this damages the tissues and organs. Autoimmune diseases occur when the immune system abnormally reacts and attacks the healthy normal cells and tissues of the body, which leads to swelling and narrowing. Over time, the inflammation can cause organ damage and serious complications. Vasculitis cannot be cured, but symptoms can be reduced and, with proper treatment, can prevent tissue and organ damage.