With an elusive etiology, vasculitis are inflammation of blood vessels, often with ischemia, necrosis, and organ inflammation, wich can affect any type of vessel—arteries, arterioles, veins, venules, or capillaries.
Primary or secondary systemic vasculitis can lead to multiorgan dysfunction, can be refractory to treatment and fatal.
As a result of inflammation in blood vessels, bleeding caused by the breakdown of vascular structures (secondary to inflammation) or interruption of blood flow can lead to organ dysfunction. This occurs in the local area of the vasculitic lesion as well as in the visceral organs and interstitium in the peripheral perfusion area.
Primary vasculitis due to inflammation of the blood vessels or secondary ones to connective tissue diseases, tumors, infections or drug allergy may lead to kidney, cardiac, cerebral, mesenteric, skin etc damage, the diagnosis and treatment of these complications involving a multidisciplinary medical team.
Clinical manifestations of vasculitic disorders are miscellaneous and depend on the size and location of the involved vessels, the extent of the organ involvement, and the degree and pattern of inflammation. From the neurological point of view, they determine polymorphic neurological manifestations.
Also, corticotherapy and immunosuppressive therapy can determine infectious complications in these patients.
Treatment of vasculitis depends of the etiology, of it’s the type and extent and severity of disease and also involve a medical team, with the purpose to induce and maintain remission.
Remission is induced by using cytotoxic immunosuppressants and high-dose corticosteroids until remission occurs or disease activity is acceptably reduced. The duration of remission is hard to predict and may depend on the type of vasculitis. For many patients, maintaining remission requires continuation of immunosuppressive therapy with or without a low dose of corticosteroids. During this period, the goal is to eliminate corticosteroids or reduce their dose and to use less potent (and less toxic) immunosuppressants as long as needed.
All patients treated with immunosuppressants should be monitored for opportunistic and other infections. Testing for tuberculosis and hepatitis B, which can become reactivated by some immunosuppressive therapies, should be done.
Clinical practice guidelines for diseases associated with vasculitis syndrome are permanently evolving.
The clinicians has to understand the diagnostic process and the clinical signs of the vasculitis syndrome and to work as a team to chose the adequate paraclinical examinations and the best treatment for the patient.