Disease characterized by inflammation of blood vessels, in which its wall is invaded by cells of the immune system.

What are Vasculitides?

The term „vasculitis” means inflammation of blood vessels. In Vasculitis, the blood vessel wall is invaded by cells of the immune system causing stenosis, occlusion, formation of aneurysms and / or hemorrhages. All organs of the body are supplied with blood through the vessels, therefore, the decrease in blood supply, due to changes in the vessels caused by Vasculitis, can cause symptoms or injuries in any system. For this reason, Vasculitides cover a diverse group of conditions.

What are Primary Vasculitis?

Primary Vasculitis are rare diseases, of little known cause, and occur when the blood vessel is the main target of the disease. The name and classification of Primary Vasculitis depends mainly on the size of the blood vessel that is usually affected. Among Primary Vasculitis, two groups are differentiated. In the first, vasculitis occurs in isolation in only one organ or tissue. This group includes vasculitis isolated from the central nervous system, skin, eyes, etc. In the second group, vasculitis affects several organs or systems simultaneously or sequentially (Systemic Vasculitis). They are: Takayasu’s arteritis and Giant Cell Arteritis or Temporal Arteritis (vasculitis that preferentially affect larger vessels); Nodose polyarteritis and Kawasaki disease (vasculitis that mainly affects medium-sized vessels); Wegener’s granulomatosis, Churg-Strauss arteritis, Microscopic polyangiitis, Urticaria vasculitis, Henoch-Schönlein purpura and cryoglobulinemia (vasculitis that affect small blood vessels).

What are Secondary Vasculitis?

Secondary Vasculitis are those in which the vessels are affected, but this involvement is associated with some other condition that may have a causal relationship with their inflammation. This is the case of vasculitis associated with other autoimmune diseases, infections, neoplasms, exposure to drugs, etc.

What are the clinical manifestations of patients with Systemic Vasculitis?

Patients with systemic vasculitis present, within a period of days or weeks, some general symptoms such as fever, tiredness and arthralgia. They may also present symptoms that will vary according to the organ (and type of vessel) affected. Skin involvement with the formation of nodules and livedo reticularis (purplish lines on the skin) is more typical of Polyarteritis Nodosa. Especially in Polyarteritis Nodosa and Takayasu’s Arteritis, important Arterial Hypertension due to renal impairment can be observed. In Wegener’s granulomatosis, Churg-Strauss syndrome and microscopic polyangiitis, renal impairment can lead to loss of function and the need for dialysis. Neurological signs and symptoms are most frequently seen in Polyarteritis Nodosa and Wegener’s Granulomatosis. Also in Wegener’s granulomatosis, the first symptom may be that of recurrent sinusopathy, but otorhinolaryngological impairment is also observed mainly in Churg-Strauss syndrome. In Temporal Arteritis, the main clinical symptom is sudden onset headache in the temporal region, sometimes accompanied by thickening of the temporal artery. As we can see, the symptoms can be very varied, and although we see some types of symptoms more frequently in certain diseases, they can occur in any type of Systemic Vasculitis.

How is the treatment of Primary Systemic Vasculitis?

At the present time, there is still no cure for Primary Vasculitis. The treatment is long-term and aims to control the disease as soon as possible (induction therapy) and maintain this control over the long term (maintenance therapy). The consultations should be frequent and regular, to evaluate the control of the disease and the possible side effects of the drugs that are being used. The course of these diseases is relatively unpredictable. Remission can be long lasting, but chronic maintenance treatment is often required. Remission periods can be interrupted without an obvious cause.