Cerebral Vasculitis

Abstract

Vasculitis is a group of vascular inflammatory diseases characterized by inflammatory and fibrinoid necrosis of the vessel wall as the main pathological feature, which can involve vessels of any size. In recent years, with the increase in the number of clinical tissue biopsy cases, there has been an increase in the number of affected vessels, tissues, types of infiltrating cells in the tissues, detectable autoantibodies in the serum, and vasculitis. The understanding of other relevant factors causing vasculitis has led to a continuous expansion and updating of the clinical classification of vasculitis. The 2012 Chapel Hill Conference further refined the understanding of vasculitis and updated the classification of vasculitis, which is currently the most widely used classification. In this classification, vasculitis has been classified into seven main categories, known as large vessel vasculitis (LVV) (includes Takayasu arteritis (TAK) and giant cell arteritis (GCA)), medium vessel vasculitis (MVV) (includes polyarteritis nodosa (PAN) and Kawasaki disease (KD)), small vessel vasculitis (SVV) (includes antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis microscopic polyangiitis (MPA), granulomatous polyangiitis (GPA)/Wegener’s granulomatosis (WG), eosinophilic granulomatous polyangiitis (EGPA)/Churg–Strauss syndrome (CSS), immune complex small vessel vasculitis, anti-glomerular basement membrane disease, cryoglobulinemic vasculitis (CV), IgA vasculitis (IgAV), and hypocomplementemic urticarial vasculitis (HUV)/anti-C1q vasculitis), variant vasculitis (VVV) (includes Behcet’s disease (BD), Cogan’s syndrome (CS)), single organ vasculitis (SOV) (cutaneous leukocytoclastic vasculitis, cutaneous arteritis, primary central nervous system vasculitis, isolated aortitis), vasculitis associated with systemic diseases (includes lupus vasculitis, rheumatoid vasculitis, and sarcoidosis vasculitis), and vasculitis associated with possible causes (includes hepatitis C virus-associated cryoglobulinemic vasculitis, hepatitis B virus-associated vasculitis, syphilis-associated aortitis, drug-associated immune complex vasculitis, drug-associated ANCA associated vasculitis, tumor-associated vasculitis, and other).