Review article: Progress of treatment in ANCA‐associated vasculitis

Abstract

This study reports on innovations in the field of, and current approaches to, the therapy of ANCA-associated vasculitis (AAV). Randomized clinical trials and prospective open label trial of newer therapies performed in the last 15 years in Wegener's granulomatosis and microscopic polyangiitis or both (AAV) were reviewed. Although cyclophosphamide remains the favoured immunosuppressive for remission induction, the use of alternative immunosuppressives and of intravenous pulsed administration have reduced cyclophosphamide exposure and are likely to increase the safety of treatment. Mycophenolate mofetil, leflunomide and deoxyspergualin are newer immunosuppressive drugs which have been evaluated in AAV, while tumpur necrosis factor, alemtuumab and rituximab are 'biologic' agents that have received attention. There is insufficient study of the dosing of glucocorticoids. Plasma exchange is indicated for severe renal vasculitis. Outcomes for elderly patients presenting with severe renal impairment are often poor despite optimal therapy. The development of collaborative networks in Europe and the USA has facilitated the conduct of larger randomized controlled trials. These have led to consensus recommendations on how AAV should be treated. Many newer agents are currently under evaluation which have the potential to improve AAV outcomes in the future.

Rituximab as Maintenance Therapy for Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis

“…Several patients developed vague symptoms such as fatigue, aches, and malaise with steroid cessation, raising a differential diagnosis of mild secondary adrenal insufficiency versus low-grade AAV-in each case, symptoms resolved with reinitiation of low-dose prednisone. To date, insufficient data remain regarding dosing and duration of steroid therapy in AAV (28). None of the clinical flares were preceded by a rising ANCA titer or repopulation of circulating B cells.…”

Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Large Vessel Vasculitis and Kawasaki Disease in Japan

“…On the other hand, randomized clinical trials of new therapies for AAV have been performed in Europe and the USA. On the principle that more severe disease requires more aggressive therapy, EUVAS devised a system for subgrouping AAV presentations, for example, based on the severity of renal impairment, for different regimens [44] . Results of such trials are very informative and might lead to consensus recommendations on how to AAV/PRV should be treated depending on the types, the disease severity and age.…”

The Association between Serological Biomarkers and Primary Sjogren’s Syndrome Associated with Peripheral Polyneuropathy

“…p-ANCA is a serum autoantibody, which could be detected in vasculitis [22], systemic lupus erythematosus [23], and SJS [24]. Several lines of evidences had demonstrated that p-ANCA is associated with vasculitic polyneuropathy, as we did in this cohort study [25–27].…”