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Treatment of ANCA-Associated Vasculitis in Adults

Aurore Viviane Fifi-Mah, Cheryl C.M. Barnabe

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Abstract

Vasculitis is the general term used to describe diseases associated with inflammation of the blood vessels. This inflammation results in end-organ ischemia and damage with lifethreatening consequences. Treatment is tailored to the type of vasculitis the patient has, prognostic features and disease severity. Two main treatment phases are recognized: induction of remission, and maintenance of remission. In this chapter we will focus on the treatment of ANCA-associated vasculitis (AAV), namely: Granulomatosis with polyangiitis (GPA), formerly Wegener’s Granulomatosis; Eosinophilic granulomatosis with polyangiitis (EGPA), formerly Churg-Strauss Syndrome, and Microscopic Polyangiitis (MPA). Patients with Polyarteritis nodosa (PAN) were included in the initial therapeutic trials of these diseases, therefore some of the studies results have been applied to that population as well.

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Vasculitis is the general term used to describe diseases associated with inflammation of the blood vessels. This inflammation results in end-organ ischemia and damage with lifethreatening consequences. Treatment is tailored to the type of vasculitis the patient has, prognostic features and disease severity. Two main treatment phases are recognized: induction of remission, and maintenance of remission. In this chapter we will focus on the treatment of ANCA-associated vasculitis (AAV), namely: Granulomatosis with polyangiitis (GPA), formerly Wegener’s Granulomatosis; Eosinophilic granulomatosis with polyangiitis (EGPA), formerly Churg-Strauss Syndrome, and Microscopic Polyangiitis (MPA). Patients with Polyarteritis nodosa (PAN) were included in the initial therapeutic trials of these diseases, therefore some of the studies results have been applied to that population as well.

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Available abstract

Vasculitis is the general term used to describe diseases associated with inflammation of the blood vessels. This inflammation results in end-organ ischemia and damage with lifethreatening consequences. Treatment is tailored to the type of vasculitis the patient has, prognostic features and disease severity. Two main treatment phases are recognized: induction of remission, and maintenance of remission. In this chapter we will focus on the treatment of ANCA-associated vasculitis (AAV), namely: Granulomatosis with polyangiitis (GPA), formerly Wegener’s Granulomatosis; Eosinophilic granulomatosis with polyangiitis (EGPA), formerly Churg-Strauss Syndrome, and Microscopic Polyangiitis (MPA). Patients with Polyarteritis nodosa (PAN) were included in the initial therapeutic trials of these diseases, therefore some of the studies results have been applied to that population as well.

Key concepts: ANCA-Associated Vasculitis, Medicine, Vasculitis, Dermatology, Internal medicine, Disease

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