Paediatric Rheumatology, Dermatology, Rheumatology

Vasculitis

Introduction to Vasculitis

Vasculitis is a term that refers to inflammation (-itis) of the blood vessels. There are many different types of vasculitis that affect different types and sizes of vessels in different locations.

Written by Doctify Team 27/04/2020

What is Vasculitis?

Vasculitis is a term that refers to inflammation (-itis) of the blood vessels. There are many different types of vasculitis that affect different types and sizes of vessels in different locations throughout the body. It is an autoimmune condition which means the body attacks itself by mistake, thinking that its own tissue is foreign. When the blood vessels are attacked, the become inflamed and damaged, resulting in occlusion and/or bleeding as the walls become weakened. There may be more significant damage if the larger vessels are affected compared to smaller, less significant vessels. Vasculitis conditions are categorised by the size of the vessels which are affected in the disease process.

What causes Vasculitis?

Vasculitis is an autoimmune condition and the reason why it occurs is not fully known. Certain genetics can make a person more susceptible and the presence of other autoimmune conditions can also predispose to developing vasculitis. The body’s immune system attacks its own vessels which causes damage that leads to occlusion and/or bleeding from the damaged vessel. This autoimmune condition can sometimes be triggered when certain susceptible patients experience certain infections or are exposed to other environmental triggers.

What are the symptoms of Vasculitis?

The symptoms vary greatly depending on what vessels are affected and at what severity. The symptoms are generally of ischaemia (reduced blood flow). Patients often present with a fever, headache, tiredness, reduction in weight, malaise, rashes, changes in sensation, night sweats and cold limbs which may not have a pulse. Depending on the specific syndrome or disease, various symptoms will be present. These include an aching jaw, vision loss and a tender scalp in giant cell arteritis and stiffness and pain in the hips, neck and shoulder regions in polymyalgia rheumatica, etc. Doctors will take a full history and perform an examination and order some investigations to find out which type of vasculitis may be present in order to treat the condition.

How is Vasculitis treated?

The treatment in vasculitis intends to primarily induce remission and then maintain that remission. Medications that suppress the immune system (immunosuppressants) are used to prevent further attack on the vessels by the body. These medications include high-dose steroids (e.g. prednisolone) and other immunosuppressant drugs such as methotrexate and cyclophosphamide. Some of these medications have side effects which the Doctor will inform you of and monitor. In severe cases, new antibody medications can be used which are generally more specific and more effective at inducing remission and controlling the symptoms. To maintain remission, treatment is altered to a reduced dose of drugs that continue to suppress the immune system (e.g. low dose steroids). Drugs such as methotrexate and cyclophosphamide are stopped as they are not suitable for maintenance therapy due to their side effect profiles and relative toxicity. Doctors will closely monitor progress through blood tests and other investigations and make any changes to medications that are necessary. Hospital admission may be necessary for severe first presentations and flare-ups, otherwise outpatient clinics and GPs can monitor progress.

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