Vaskuliten

Vaskuliten, or vasculitis, is a collective term for a group of disorders characterized by inflammation of the walls of blood vessels. The inflammation can affect arteries, veins, and capillaries of any size, potentially leading to narrowed or weakened vessels, reduced blood flow, organ damage, or aneurysm formation. The condition can be limited to a single vessel or involve multiple organs, and it may be acute or chronic.

Classification generally follows vessel size. Large vessel vasculitis includes conditions such as giant cell arteritis and Takayasu arteritis. Medium vessel vasculitis encompasses disorders like polyarteritis nodosa and Kawasaki disease (the latter primarily affecting children). Small vessel vasculitis includes several immune-mediated syndromes, notably ANCA-associated vasculitis (granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis) and IgA vasculitis. Each type has distinct patterns of organ involvement and prognosis.

Etiology is diverse and often immune-mediated, with triggers including infections, drugs, or underlying autoimmune diseases. In many cases the exact cause remains unknown. Diagnosis relies on a combination of clinical features, laboratory tests (such as inflammatory markers and, when appropriate, autoantibodies like ANCA), imaging studies, and tissue biopsy of affected vessels or organs.

Treatment aims to control inflammation, prevent organ damage, and manage complications. First-line therapy typically involves corticosteroids, with additional immunosuppressants or targeted biologic agents used for certain types. Management requires ongoing monitoring for relapse and treatment-related adverse effects. Outcome varies by type and extent of organ involvement but has improved with earlier recognition and therapy.