Vasculitides in autoimmune diseases are an important cause of secondary headaches. The article discusses the incidence of headache in primary and secondary vasculitides of the central nervous system. The symptoms of primary CNS vasculitis are presented. The occurrence of headache in large-vessel vasculitides, such as Takayasu arteritis and giant cell arteritis; medium-vessel vasculitides, such as polyarteritis nodosa and Kawasaki disease; and small-vessel vasculitides, such as microscopic polyangiitis, granulomatosis with polyangiitis, and eosinophilic granulomatosis with polyangiitis, are characterized. The occurrence of headache in vasculitides of different blood vessels, such as Behcet's disease and Cogan's syndrome, is presented as well. Systemic autoimmune diseases discussed in the paper are systemic lupus erythematosus, antiphospholipid antibody syndrome, rheumatoid arthritis, Sjogren's syndrome, and sarcoidosis. Vasculopathies which can mimic CNS vasculitides were discussed as well. Examples include reversible cerebral vasoconstriction, Susac's symdrome, and cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL).
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