Vasculitis: symptoms and therapies

They are a group of diseases, rare if taken individually, but not to be underestimated as a whole.

Vasculitis are diseases in which an inflammatory process occurs in the blood vessels, both those of small caliber, such as capillaries and arterioles, and those of medium and large caliber. More often only the arteries are affected, while only in some rarer conditions are the veins also involved.

Vascuilitis can be a complication of other diseases (tumors, infections, autoimmune diseases such as rheumatoid arthritis or lupus), but more often they are primary vasculitis, which constitute a large and heterogeneous group of diseases: among the most frequent we remember:

• Horton’s gigant-cellular arteritis
• Wegener’s granulomatosis
• Churg-Strauss syndrome
• Behçet syndrome.

They are autoimmune diseases

At the base of primitive vasculitis there is an autoimmune mechanism: the immune system is activated against blood vessels, resulting in chronic inflammation. Why this happens, however, has not yet been discovered.

What is certain is that more factors come into play, starting with genetic susceptibility: recently for each vasculitis some alterations have been identified in specific genes that favor a greater predisposition towards the disease.

In subjects genetically predisposed to vasculitis, other factors come into play that can trigger the autoimmune reaction; In particular, it is hypothesized that infectious agents, such as viruses and bacteria, and also exposure to environmental elements such as chemicals, drugs and food may play a role.

Very varied symptoms

Vasculitis is united by the presence of inflammation and necrosis of the walls of blood vessels of any caliber and in any part of the body. Inflammation of the vessels can cause obstructions (stenosis and possible thrombosis) or dilations (aneurysms) of the same.

The symptoms, however, can be the most varied. Some are common to various types of vasculitis, and are generally the most non-specific, such as fever, malaise, joint pain, fatigue.

The most peculiar manifestations, which can help in diagnosis, are different depending on the type of vasculitis and can manifest themselves differently even among subjects affected by the same disease, complicating the diagnosis. For example, in Horton’s giant cell arteritis, the first symptom is often a very strong headache, which arises suddenly in those who did not suffer from it before, in most cases in the frontal and temporal area of the head, while in Behçet’s disease, the first sign is often the appearance of canker sores in the mouth, frequently associated also with genital ulcers.

With variable frequency depending on the type of vasculitis, in addition to blood vessels, other parts of the body may be involved (in particular kidneys, heart, lungs, eyes, central nervous system, peripheral nerves, intestine).

How to deal with each other

Early diagnosis and treatment now allow us to live with vasculitis, leading an almost completely normal life.

The diagnosis, however, is not easy, because there are no specific blood tests that allow the identification of the disease; However, there are some markers that can be analyzed and that differ depending on the type of vasculitis. If the disease is suspected, additional tests (such as PET, CT scans or biopsies) may be prescribed, from which to choose based on the type of vasculitis hypothesized.

As for therapies, it generally starts with high-dose corticosteroids (such as prednisone), useful for immediately turning off inflammation, followed by the administration of immunosuppressants, drugs that block the multiplication of cells involved in the autoimmune reaction. Classical immunosuppressants (such as methotrexate, azathioprine) are generally used, but, where possible, the latest generation ones are also used, the so-called monoclonal antibodies (such as rituximab, tocilizumab).

However, the therapy must be personalized in each patient and according to the type of vasculitis. Generally the treatments continue for a few months or years, they can be suspended when the disease is in remission (ie when the symptoms disappear), but it may be necessary to have to resume them when the manifestations reappear, to keep the disease under control.