Vasculitis syndrome-diagnosis and therapy

Abstract

In patients with connective tissue disease, vascular injury induced by primary or secondary vasculitis syndromes can lead to organ dysfunction due to the loss of nutrient supply from the blood. Such vasculitis syndromes can be refractory to treatment and fatal. The nomenclature and the definition of vasculitis syndromes have recently been revised, and clinical practice guidelines for diseases associated with vasculitis syndrome are evolving. The present review provides an overview of vasculitis syndromes from the viewpoint of diagnosis and treatment.

Keywords: diagnosis; nomenclature; therapy; vasculitis syndrome.

Figure 1

Approach to the diagnosis of vasculitis syndrome. TAK, Takayasu arteritis; GCA, giant cell arteritis; PAN, polyarteritis nodosa; MPA, microscopic polyangiitis; EGPA, eosinophilic granulomatosis with polyangiitis; GPA, granulomatosis with polyangiitis; aGBM, antiglomerular basement membrane disease; IgAV, IgA vasculitis; CV, cryoglobulinemic vasculitis; RV, rheumatoid vasculitis. Revised from reference 3. Kawasaki disease is excluded in this diagnostic procedure because the diagnostic procedure for Kawasaki disease is based solely on characteristic clinical signs and symptoms

Figure 2

Protocol for medical treatment of Takayasu arteritis. PSL, prednisolone; MTX, methotrexate; PO, oral administration; IV, intravenous injection; CsA, cyclosporin A; AZP, azathioprine; MMF, mycophenolate mofetil. Revised from reference 3

Figure 3

Induction therapy for remission in polyarteritis nodosa. Revised from reference 3. mPSL, methylprednisolone; PSL, prednisolone; IVCY, intravenous cyclophosphamide; CY, cyclophosphamide