David R W Jayne

..Regimens involving maintenance phases have been developed, but uncertainty remains about the risk of flares and how they can be predicted. The optimum duration of treatment has yet to be determined...

..Further treatment with cyclophosphamide achieved a second remission, but the patient relapsed again despite remission-maintaining treatment with mycophenolate mofetil...

..Treatment with rituximab has led to remission rates of 80 to 90% among patients with refractory ANCA-associated vasculitis and may be safer than cyclophosphamide regimens...

..Individualized evaluation of risk and treatment selection will help maximize effectiveness and minimize toxicity...

..Improvement in renal outcomes largely relies on the evaluation of more selective agents in carefully controlled studies...

..The toxicity and infective risk of B cell-targeted agents in renal disease needs to be determined as well as their optimal dosing in combination with conventional agents...

..The presentation and management of renal involvement in microscopic polyangiitis is discussed, with emphasis on prognosis and outcomes. Potential roles of newer therapies are reviewed...

..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...

..When the diagnosis remains uncertain, observation over time, repeat investigation and a therapeutic trial may improve the probability of the diagnosis or identify an alternative disease...

..Patient survival and severe adverse event rates were similar in both groups...

..Their correct evaluation and integration with current regimens to improve long-term outcome is the major challenge in clinical vasculitis research today...

..An understanding of the biology of MPO and its involvement in the pathogenesis of vasculitis is of importance in understanding the pathogenesis of vasculitis and the development of newer therapies...

..With the availability of other alternative therapies in refractory disease, there needs to be a clear demonstration of the benefits of HSCT from current randomized trials...

..The safety of this procedure is likely to be improved by patient selection and choice of conditioning regimen. The return of disease activity in one-third of patients might be reduced by long-term immunosuppressive therapy post-ASCT...

..These subgroups, which are also found in the normal population and can be identified by measuring expression of only three genes, raise the prospect of individualized therapy and suggest new potential therapeutic targets in autoimmunity...

..This permitted comparison of rituximab dosing regimens, the value of continuing immunosuppression, and investigation of ANCA and B cell levels as re-treatment biomarkers...

..However, further relapse is common, and maintenance strategies are required. The aim of this study was to reduce relapse rates using a fixed-interval rituximab re-treatment protocol...

..To perform a literature review and develop recommendations for the use of rituximab in ANCA-associated vasculitis...

..Renal involvement is frequently present in antineutrophil cytoplasmic autoantibody (ANCA)-associated systemic vasculitis and is an important cause of end-stage renal failure (ESRF)...

..Current remission maintenance therapies for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are limited by partial efficacy and toxicity...

..The complexities of clinical trials in lupus nephritis have impeded the demonstration of the efficacy of new agents, but if these difficulties can be overcome, there is a real chance that outcomes in lupus nephritis will improve...

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..However, longer follow-up indicates a subsequent relapse rate of over 50% that may be associated with low MMF dosing...

..The hemophilia followed a relapsing course under oral corticosteroid therapy, but B-cell depletion with anti-CD20 monoclonal antibody (rituximab) was effective in inducing a prolonged remission...

..This study aimed to evaluate the response of refractory Wegener's granulomatosis affecting the ear, nose and throat and granulomatous eye disease to B-cell depletion with rituximab...

..Its cause is unknown, and there is debate about whether it is a single disease entity and what role ANCA plays in its pathogenesis. We investigated its genetic basis...

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..Larger, randomised comparisons to standard therapy are needed along with assessment of long-term efficacy and safety...

..As rare diseases, treatment advances in vasculitis depend on international collaborative research networks both to establish an evidence base for newer agents and to develop recommendations for patient management...

..Humanised monoclonal anti-CD52 antibodies (alemtuzumab, CAMPATH-1H) selectively deplete lymphocytes. We present long-term follow-up results of patients with relapsing/refractory AASV treated with CAMPATH-1H...

..Future randomized control trials and long-term follow-up studies are required to confirm the role of RTX and other B-cell targeting therapies in AASV and SLE...

..The established infrastructure and accumulated database are well placed to facilitate testing of newer therapeutic strategies in the future...

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..The possible differences in histopathology are investigated in this study...

..Even with ominous histologic findings, the chance of renal recovery exceeds the chance of therapy-related death when these patients are treated with plasma exchange as adjunctive therapy...

..36, P = 0.01). Normal glomeruli were a positive predictor of dialysis independence and improved renal function after 12 mo, indicating that the unaffected part of the kidney is vital in determining renal outcome...

..This unblinded, prospective, randomized, controlled trial was undertaken to determine whether methotrexate (MTX) could replace CYC in the early treatment of AASV...

..Cyclophosphamide-associated neutropenia and infection were frequent contributors to death, and less toxic alternatives may improve outcome in PRS...