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Vasculitis Syndrome - Symptoms, Causes, and Treatment

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Vasculitis is an auto-immune disease that causes the inflammation and narrowing of blood vessels of all sizes. Read the article

Medically reviewed by

Dr. Kaushal Bhavsar

Published At September 11, 2023
Reviewed AtSeptember 11, 2023

What Is Vasculitis?

Vasculitis is the inflammation of all the body's blood vessels ranging from the smallest (capillaries) to medium-sized and the largest (the aorta-main blood vessel that leaves the heart).

Due to the inflammation, these blood vessels become stretched, weakened, and lead to aneurysms (bulges in the wall of the blood vessel). The blood vessels become so thin that they may rupture, and the blood flows into the tissues. It causes a range of symptoms whose severity depends on the affected part. Some affected parts include-

  • Skin: Spots/rashes in the skin seen. These sports can burst to leave open ulcers.

  • Nose: Nosebleeds and crusting inside the nose occur.

  • Eyes: Vision becomes unclear sometimes.

  • Nerves: Nerve inflammation can cause pain and weakness in the arms and legs.

  • Lungs: Inflammation of the lung causes coughing or shortness of breath.

  • Brain: The brain's blood vessels are sometimes involved, causing issues like stroke.

  • Kidneys: Dark-colored or bloody urine is seen.

Some other common symptoms include- fever, fatigue, joint pain, weakness, etc.

What Is Vasculitis Syndrome?

Systemic vasculitis can be divided into two types

  • Primary Vasculitis Syndrome:

It is commonly caused due to the inflammation of the blood vessels. It is a systemic auto-immune disorder.

  • Secondary Vasculitis Syndrome:

It is caused due to other conditions such as connective tissue diseases, drug allergies, tumors, other infections, etc. It is a systemic auto-immune disorder.

Vascular injury induced by primary or secondary vasculitis syndrome in patients with connective tissue disease can lead to organ dysfunction due to the loss of nutrient supply from the blood. These vascular syndromes are difficult to treat and can be fatal.

What Are the Symptoms Associated With Vasculitis Syndrome?

The symptoms associated with vasculitis syndrome depend on the affected blood vessel and the organ to which it supplies the blood. Some of the examples of affected blood vessels and associated symptoms are

  • Ophthalmic Artery: Blurred vision or blindness in some cases.

  • Maxillary Artery: Jaw claudication.

  • Subclavian Artery: Numbness or cold sensation in the upper extremities, the difference in the blood pressure between the left and right arms, and pulse deficit.

  • Renal Artery: Hypertension and renal failure

  • Coronary Artery: Angina pectoris, myocardial infarction.

  • Pulmonary Artery: Coughing or shortness of breath.

Some other symptoms include-

  • Skin rashes are often called palpable purpura in the lower extremities mainly.

  • High-grade fever.

  • Weight loss is also seen due to inflammation.

  • Weakness or malaise is caused.

  • Joint pain occurs due to insufficient blood supply to the organs.

  • Heart attack- if the blood vessel supplying the heart is affected.

  • Brain stroke- if the blood vessel supplying blood to the brain is affected.

What Causes Vasculitis Syndrome?

The exact cause cannot be determined. However, it is seen where the immune system becomes overactive and ends up attacking parts of the body. In most cases of vasculitis, something causes an immune or allergic reaction in the walls of blood vessels. Substances that cause allergic reactions are called antigens. Sometimes certain medicines or illnesses can act as antigens and result in vasculitis.

How Is Vasculitis Syndrome Diagnosed?

It can be diagnosed based on affected blood vessels or organs in many ways, such as-

  • Clinical Evaluation:

After a thorough physical examination, the patient's history is asked, and signs of inflammation are checked.

  • Some laboratory tests are done to check for inflammation and organ dysfunction and to determine the stage of the disease, such as CBC (Complete Blood Count), ESR (Erythrocyte Sedimentation Rate), C- reactive protein, serum albumin, complete protein, creatinine, BUN (Blood Urea Nitrogen), etc.

  • Urine Tests:

These are done to check for kidney damage. The presence of blood or protein is tested via this.

  • Imaging Techniques:

These include CT (Computed Tomography) scans, MRI (Magnetic Resonance Imaging), X-rays, and ultrasounds. These are performed to check for inflammation in the blood vessels and affected body organs.

  • Angiogram:

It is often done where abdominal organs, such as the kidney and gut, get indulged. In this technique, dye is injected into the bloodstream by doctors to check for a better picture of blood vessels

  • Echocardiogram:

This test determines the functioning of the heart. It is based on a special type of ultrasound testing.

  • Electrocardiography (ECG):

It is also advised. Electromyography (EMG) and nerve conduction studies are also done to diagnose latent vasculitis syndrome and locate an appropriate biopsy site.

  • Biopsy:

A small piece of tissue is taken from the affected blood vessel or the organ and is checked for signs of inflammation.

How Is Vasculitis Syndrome Treated?

The treatment of vasculitis should be done as soon it is diagnosed, as it becomes difficult to maintain blood flow through the affected blood vessels. It can cause serious harm to the blood vessels involved and result in the dysfunction of associated organs. The treatment relies on the blood vessels and organs involved. The treatment of vasculitis is as follows-

  • Medications:

Corticosteroids are the choice of drugs in treating vasculitis, enormous vessels. It is a life-saving drug that has got strong anti-inflammatory properties. Doctors administer steroids in the initial stage of the disease. Prednisolone is considered an important drug in this regime. The dose and duration of medication depend on the severity of the disease. In long-term use, they can have side effects.

  • Immunosuppressants:

These drugs are administered to reduce inflammation immediately. Also, some people are resistant to corticosteroids. These drugs are the only choice. For example- Cyclophosphamide is a drug used in treatment. Therefore, newer methods are also being brought up with the help of immunosuppressants to control the disease in corticosteroid-resistant vasculitis. Their side effects are less than corticosteroids.

  • Surgery:

It is done in those cases where the damage from vasculitis is severe.

Vascular bypass grafting is done, which helps in the redirection of blood flow around a blockage in the blood vessel. Depending on the damage location, other surgeries are done, such as sinus surgery or kidney transplant.

Conclusion

This article gives details of vasculitis syndrome, its type, symptoms, and possible treatment. Vasculitis is the inflammation of blood vessels in any part of the body. It is a rare disease but can be life-threatening if the involved blood vessel is of any vital organ such as the heart or brain. It is an auto-immune disorder. Vascular injury, when induced by primary or secondary vasculitis syndrome in patients of connective tissue disease, can lead to organ dysfunction as the nutrient supply from the blood becomes lost. Such vasculitis syndrome can be serious and fatal. It can result in various symptoms depending on the associated organ, such as skin rashes, joint pain, abdominal pain, brain stroke, heart attack, etc. The choice of drug for the treatment of Vasculitis is Corticosteroid. Its strong anti-inflammatory property makes physicians administer it early in life.

Dr. Kaushal Bhavsar
Dr. Kaushal Bhavsar

Pulmonology (Asthma Doctors)

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