Vasculitis: Causes, Types, Symptoms, Diagnosis, and Complications

Introduction

Vasculitis is a group of pathologies characterized by the inflammation of blood vessels in the body. All diseases share some common features, including clinical, laboratory, and pathophysiologic features. However, the clinical and pathological features may vary and depend on the site and type of blood vessels affected.

What Is Vasculitis?

Vasculitis means inflammation of blood vessels that carry the blood around the body. There are three types of blood vessels -

• Arteries - It takes blood from the heart to different body parts, such as organs like kidneys and tissues like skin.

• Veins - These take the blood back to the heart.

• Capillaries - These are the tiny vessels between the arteries and the veins from where oxygen and nutrients are passed from the blood to the tissues.

The organs and tissues in the body require a continuous blood supply to function properly. Therefore inflammation in these vessels leads to reduced or even blockage in the blood flow, causing various complications and a range of syndromes. The amount of damage vasculitis causes depends upon the body part it damages. Moreover larger the affected blood vessels more serious the damage would be.

Vasculitis may occur suddenly on its own without any associated disorder, which is called primary vasculitis. Vasculitis occurs due to other underlying conditions, such as rheumatoid arthritis (chronic inflammatory condition of joints) or Sjogren's syndrome (immune system disorder causing dry mouth and eyes), known as secondary vasculitis.

What Causes Vasculitis?

In the majority of cases, the exact cause of vasculitis is unknown and is mostly associated with the immune system of the body. Sometimes the immune system, which works to protect the body, gets triggered or becomes overactive due to some allergic reaction and starts to attack the healthy parts of the body. This can lead to inflammation in the walls of blood vessels. Sometimes certain medications, diseases, or foreign substances can trigger the immune system known as an antigen. Therefore, most vasculitis diseases are types of autoimmune diseases which means that the immune system of the body is not working properly and is attacking the healthy parts of the body instead of fighting infections.

What Are the Symptoms of Vasculitis?

Vasculitis causes inflammation of blood vessels leading to pain, which cannot be viewed clinically as the inflammation is inside the body. Patients with vasculitis usually feel unwell and have a fever, fatigue, sweats, and weight loss. These are usually the first signs of vasculitis a person experiences. Other symptoms of vasculitis may vary depending upon the part of the body affected, which include the following -

• Skin - Vasculitis in the skin leads to spots that burst with time, leaving behind open sores (ulcers). These are not serious and get resolved with time.

• Fingers and Toes - Some people experience Raynaud's phenomenon in which the toes and fingers turn blue or white and may cause pain when exposed to cold conditions.

• Nose - Vasculitis leads to dryness or crusting inside the nose leading to bleeding, and in some cases, the shape of the nose may also get changed.

• Eyes - Vasculitis can affect eyesight or cause the eyes to become red or painful. In this case, it is very important to see a doctor.

• Joints - Can cause joint pain or swelling.

• Muscles - Inflammation of vessels can lead to muscle aches and may cause muscle weakness.

• Nerves - Inflammation of nerves can lead to pain and tingling or burning sensation in arms and legs.

• Lungs - This can affect the efficiency of the lungs and lead to shortness of breath and coughing.

• Brain - Vasculitis can cause an interruption in blood supply to the brain or bursting of blood vessels in the brain, known as a stroke or brain attack.

• Kidneys - Vasculitis can lead to problems in passing urine or blood in the urine. This a serious problem that only appears when the kidneys get damaged.

What Are the Types of Vasculitis?

The types of vasculitis can be classified according to the type of vessel included, such as a large, medium, or small vessel. Therefore there are various types of vasculitis which include -

1. Giant Cell Arteritis (Temporal Arteritis) - This type usually affects the arteries in the head and neck and is mostly seen in adults over the age of 50 years. This is called temporal arthritis, as the arteries near the temple are mostly affected. This can cause the following:

• Pain and soreness around the temple.

• Headaches.

• Jaw muscle pain while eating.

• Vision loss or double vision.

This type mostly occurs with polymyalgia rheumatica and can be treated with steroids.

2. Polymyalgia Rheumatic - This type is mostly seen in adults over 50 years and is more common in women than men. It is strongly associated with temporal arteritis and can lead to -

• Pain and stiffness in the shoulders, neck, and hips.

• High fever.

• Depression.

• Tiredness.

• Loss of appetite and weight loss.

This condition can be treated with steroids.

3. Takayasu Arteritis - This usually affects young women and is a rare condition, which usually affects the main artery of the heart as well as the other major arteries of the heart. It can lead to -

• High fever.

• Extreme tiredness.

• Muscle and joint pain.

• Shortness of breath.

• Pain in limbs.

• Weight loss.

• Cold or numb limbs.

Treatment is done through steroid medication.

4. Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome) - This type is usually seen in adults aged 30 to 45. It can lead to conditions like -

• Asthma.

• High fever.

• Cold-like symptoms.

• Tiredness.

• Weight loss.

• Muscle and joint pain.

In addition, it can also affect the nerves causing weakness and numbness in extremities and damaging the kidneys and heart muscles in severe cases. It is usually treated with steroid medicines.

5. Granulomatosis With Polyangiitis (Wegener’s Granulomatosis) - This condition mainly affects the blood vessels of the nose, ear, lungs, kidneys, and sinuses. It mainly affects middle-aged or older adults. The following signs and symptoms can be seen in this, which include:

• High fever.

• Sinusitis (inflammation of sinuses).

• Night sweats.

• Shortness of breath and coughing up blood.

• Nosebleeding.

• Kidney problems.

This serious condition can lead to organ failure and death if left untreated. Therefore, it is mainly treated with medicines such as immunosuppressants and steroids.

6. Kawasaki Disease - This condition mainly affects children under the age of five, and the main characteristic features of this disease include the following:

• High fever that lasts for more than five days.

• Dry or cracked lips.

• Red eyes.

• Skin rash.

For this condition, intravenous immunoglobulin (IVIG), a solution of antibodies, and Aspirin can be given. However, Aspirin is contraindicated in children under 16 years of age.

7. Polyarteritis Nodosa - A rare vasculitis usually affects the arteries supplying the kidneys, gut, and nerves. It usually appears in childhood or middle-aged people and can be triggered by an infection such as hepatitis B. It can cause the following symptoms:

• Muscle and joint pain.

• Bleeding in the gut.

• Abdominal pain.

It can be serious if not treated on time and can be treated by steroids or immunosuppressive drugs.

8. Behcet's Syndrome - This rare autoimmune condition involves mouth ulcers, genital ulcers, and skin and eye problems. It can also involve other body parts, such as the gastrointestinal tract and cardiovascular and neurological systems. Different medications are used to treat this condition, as various body parts are involved in this.

How to Diagnose Vasculitis?

The diagnosis of vasculitis is based on the symptoms, medical history, physical examination, and other laboratory tests. The healthcare professional can advise blood tests to check for any abnormalities such as:

• Anemia (low red blood cell count).

• High or low platelets or white blood cells count.

• Kidney or liver problems.

Blood tests show antibodies that may be associated with vasculitis can be identified. Additionally, some additional tests may also be done, such as -

• X-rays, CT (computed tomography), and MRI (magnetic resonance imaging) scans can be used to check for chest problems.

• Echocardiograms and electrocardiograms can be used to assess the condition of the heart.

• A biopsy can be done to check the conditions of the kidneys, muscles, skin, or lungs.

• Angiogram can be done in case of organs like kidneys or gut are involved.

What Are the Complications Associated With Vasculitis?

The complications of vasculitis usually depend upon the type of condition. Some of the most common complications include the following:

• Multiple organ damage.

• Blood clots and aneurysms (abnormal bulges or ballooning in the wall of vessels).

• Vision loss or blindness.

• Infections, as the medication prescribed is immunosuppressant, can make the body more prone to secondary infections.

Conclusion

Vasculitis can be a short-term or lifelong condition. The treatment usually focuses on preventing any permanent damage to the vital organs. Following the treatment therapy advised by the health care professional is crucial. A patient can live a long and happy life by following the proper treatment therapy and taking all the necessary precautions to avoid any secondary infections.