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Overview of Vasculitis

Team AckoJan 17, 2024

The term Vasculitis (VCS) refers to the inflammation of blood vessels. This word has been derived from the Latin vasculum, meaning vessel. In general, the suffix “itis” refers to inflammation anywhere in the body.

VCS is a general term used to refer to a group of rare diseases where the blood vessels supplying different organs are affected. When the blood flow to any organ is disrupted, it could result in disastrous consequences like organ failure. The symptoms of the disease are non-specific, therefore, the symptoms are highly varied. It can affect people of all ages. However, there are certain specific types of Vasculitis which occur in particular age groups (eg: Kawasaki’s disease occurs in children). Read ahead for an overview of this condition.

Contents

What are blood vessels?
What is the cause of Vasculitis?
What are the symptoms of Vasculitis?
What are the types of Vasculitis?
How is Vasculitis diagnosed?
What is the treatment of Vasculitis?
Frequently Asked Questions (FAQs)

What are blood vessels?

There are different types of blood vessels in the body. In general, they are Arteries, Veins, and Capillaries. In addition to this, based on their size, blood vessels can be classified as small, medium, and large vessels.

What is the cause of Vasculitis?

The exact cause of VCS is still not known. Generally, it is thought to be related with an auto-immune pathology.

Auto-immune diseases are those where the body’s immune system attacks its own tissues because it perceives some native component to be foreign. In VCS, the immune system recognizes some component of the blood vessels as foreign, and starts attacking it.

This results in inflammation and when left untreated can cause severe vessel damage. This causes reduced and impaired blood flow to the organs & tissues, resulting in systemic damage & symptoms.

Some types of VCS can occur following certain infections, which is especially seen in people with chronic Hepatitis B & Hepatitis C, and syphilis. Vasculitis can also be a component of other autoimmune & rheumatic conditions, such as Rheumatoid Arthritis, and SLE (systemic lupus erythematosus). Rarely certain medications can trigger this condition. It is not necessary that a person diagnosed with VCS have an underlying isolated cause.

What are the symptoms of Vasculitis?

Vasculitis can present with a wide variety of symptoms, depending on the affected organ system. Some of these symptoms include the following.

General symptoms: Fever (which may be prolonged), weight loss, general tiredness & lethargy, loss of appetite.
Skin: Skin rashes of different types can occur, usually presenting as reddish-purple discoloration of the skin, purpura, or ulcers.
Nervous system: Headache, stroke, numbness & tingling of arms and legs.
Eyes: Vision issues can range from blurring of vision to sudden loss of eyesight.
Ears: Ringing sensation in the ear (tinnitus), sudden hearing loss.
Heart: Heart attack, breathing difficulty, high blood pressure.
Respiratory system: Cough, shortness of breath, wheezing, poor lung function.
Gastrointestinal system: Abdominal pain, blood in vomit, blood in stools/black tarry stools, ulcers, perforations (which are holes formed in the GI tract resulting in life-threatening complications).
Bones & Joints: Bone and joint pain, swelling of joints, restriction of movement, difficulty in performing routine actions.
Muscles: Muscle pain, swelling, decreased functioning.
Kidneys: Decreased urine output, blood in urine.
Gangrene and death of fingers, toes & limbs.

This is a broad list of possible symptoms and does not include every possible presentation. Sometimes, certain diseases may not cause a visible symptom initially and may only present at a more severe, later stage. The range of severity also varies; while some may have a mild form of disease, some people may end up with a life-threatening condition.

What are the types of Vasculitis?

There are many ways by which Vasculitis is classified. Based on the type of blood vessel involved, the condition is classified as follows.

Large vessel Vasculitis
Medium-sized vessel Vasculitis
Small vessel Vasculitis
Small & Medium-sized vessel Vasculitis
Vasculitis affecting vessels of all sizes

There are different medical conditions in each group. Your doctor may advise a series of tests to find the exact cause.

How is Vasculitis diagnosed?

The diagnosis of Vasculitis is at times difficult because the symptoms are nonspecific and could have many causes. Your doctor will elicit a detailed history and perform a thorough physical examination to look for any signs indicative of an underlying cause.

Common investigations include the following.

Complete blood count to check for white blood cell count
Level of acute phase reactants (CRP, ESR)
Antibody levels such as ANCA
Radiological examination such as X-rays/CT scans/MRIs/PET scans
Angiogram to view blood vessels
A biopsy to examine the tissue under a microscope may be required to reach a specific diagnosis

What is the treatment of Vasculitis?

The treatment for a Vasculitis disorder is a multi-disciplinary approach involving a team of specialists usually headed by a rheumatologist. It depends on the type of the disease, the severity, as well as the organ system involved.

The treatment protocol commonly includes the following.

Steroids: They are a group of drugs that decrease inflammation, and suppress your immune system so as to counteract the auto-immunity. The choice of steroid medication, and the duration of treatment are determined based on your diagnosis, the severity of your symptoms and your other pre-existing conditions.
Steroid-sparing agents: Steroids may need to be used for a prolonged duration, and require regular follow-up & careful dose titration as they can have many side effects. Immune suppressor medications like cyclophosphamide, methotrexate, and azathioprine are also used in the treatment for different types of Vasculitis.
Newer agents: Newer medications that are used for treatment include biological agents like rituximab, tocilizumab, etc., which are designed to decrease inflammation and severity of disease.

Some complications of Vasculitis may require surgical intervention.

VCS can lead to a condition called aneurysm of blood vessels. This area is very susceptible to rupturing and causing massive bleeding. An aneurysm requires surgical intervention to prevent it from rupturing and causing danger to life.

VCS can also cause gangrene which may necessitate amputation of the toe/finger or even a part of the limb.

There are also surgical procedures where a vessel that is blocked is bypassed by using a graft to redirect the blood flow around the blockage. Vasculitis can also result in organ failure which might require transplantation to treat.

Frequently Asked Questions (FAQs)

Here’s a list of common questions and answers related to Vasculitis.

I suddenly got an itchy rash over my legs and stomach pain. What could be the reason?

There are a lot of reasons for rashes and abdominal pain. One common cause is a specific type of Vasculitis called Henoch-Schonlein purpura. Visit a rheumatologist or internal medicine specialist to diagnose the cause and give appropriate treatment.

I have been diagnosed with Vasculitis. Can I undergo any preventive measures at home?

Vasculitis can be triggered by multiple factors. Avoiding them and maintaining a healthy lifestyle with adequate sleep, nutritious diet, and regular exercise is helpful. Avoid smoking and tobacco use.

How long will I have to take tablets to treat Vasculitis?

The initial episode of Vasculitis may take a few weeks to respond to treatment. Even after that, you may have to continue medication long term in case of severe disease. Always consult your doctor before starting or stopping any medication.

Disclaimer: The content on this page is generic and shared only for informational and explanatory purposes. Please consult a doctor before making any health-related decisions. Vasculitis is written as VCS in this article on a few occasions.