Vasculitis

Vasculitis is inflammation in vessel walls leading to poor blood circulation and damage to vessels. The vasculitis are devided into large vessel vasculitis (Takayasu arteritis [TAK] and Giant cell arteritis [GCA]), medium vessel vasculitis (Polyarteritis nodosa and Kawasaki disease), small vessel vasculitis associated with antineutrophil cytoplasmic antibody (microscopic polyangiitis [MPA], granulomatosis with polyangiitis [GPA], eosinophilic granulomatosis with polyangiitis [EGPA]) and others. TAK affects patients younger than 50 years, and GCA after age 50. Clinical features include leg claudication, headaches, postural dizziness, visual disturbances and reduced or absent upper limb pulses. Management of TAK and GCA include high doses of GCS. Treatment of GCA can prevent blindness due to occlusion of optic arteries. New option is tocilizumab. Polyarteritis nodosa inflammation involves the skin, kidneys, peripheral nerves, muscles, and gut in patients between 40-60 years old. Management includes GCS, CYC and AZA. Kawasaki disease is an acute febrile mucocutaneous and lymph node disease affecting young children. Coronary arteries are often involved. Management includes IVIG and aspirin. GPA is necrotizing granulomatous inflammation involving the upper and lower respiratory tract, necrotizing glomerulonephritis, ocular vasculitis, pulmonary capillaritis with hemorrhage and granulomatous and nongranulomatous extravascular inflammation is common. c-ANCA is present in 98% of patients. Management is high doses of GCS, CYC, rituximab and AZA. EGPA is characterized by pulmonary and systemic small vessel vasculitis, granulomas and hypereosinophilia. Clinical features are atopic history, asthma, allergic rhinitis, pulmonary infiltrates, mono/polyneuropathy, mononeuritis multiplex, purpura and eosinophilia. Management is high doses of GCS, CYC and AZA.

Keywords: Antineutrophil cytoplasmic antibody, AZA, CYC, Eosinophilic granulomatosis with polyangiitis [EGPA], GCS, Giant cell arteritis [GCA], Granulomatosis with polyangiitis [GPA], Kawasaki disease, Large vessel vasculitis, Medium vessel vasculitis, Microscopic polyangiitis [MPA], Polyarteritis nodosa, Rituximab, Small vessel vasculitis, Takayasu arteritis [TAK], Vasculitis.