Abstract

Systemic necrotising vasculitis is a group of potentially serious inflammatory diseases of unknown aetiology, involving destructive inflammation of the vascular wall and mainly affecting the small and medium-sized vessels. Symptoms vary depending on the size and location of the affected vessels. Untreated, they are associated with high morbidity and mortality. Inflammation of the vascular wall causes obstruction of the vessel lumen with ischaemia and necrosis of the tissues it irrigates. We suspect the condition when several organ systems are affected simultaneously or consecutively, in the presence of a prolonged febrile syndrome or an unexplained toxic syndrome, ischaemic manifestations (skin ulcers, digital ischaemia) or other unusual clinical manifestations (mononeuritis multiplex, pulmonary infiltrates or nodules).When vasculitis is suspected, a detailed clinical history should be taken. In terms of laboratory tests, there are usually non-specific findings (anaemia, elevated ESR and C-reactive protein) that can be very striking, but do not differentiate vasculitis from other inflammatory or proliferative processes or, in many cases, renal failure. Treatment is based on the administration of glucocorticoids, combined or otherwise with immunosuppressive drugs and/or biological therapy, depending on its extent and severity.

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