***TASK*** the task is to summarize an input biomedical literature in six sentences 
    ***INPUT*** the input is a biomedical literature 
    ***OUTPUT*** the output is the summary of an input biomedical literature in six sentences 
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    Input: Systemic vasculitis associated with autoantibodies to neutrophil cytoplasmic antigens ( ANCA ) is the most frequent cause of rapidly progressive glomerulonephritis . Renal failure at presentation carries an increased risk for ESRD and death despite immunosuppressive therapy . This study investigated whether the addition of plasma exchange was more effective than intravenous methylprednisolone in the achievement of renal recovery in those who presented with a serum creatinine > 500 micromol/L ( 5.8 mg/dl ) . A total of 137 patients with a new diagnosis of ANCA-associated systemic vasculitis confirmed by renal biopsy and serum creatinine > 500 micromol/L ( 5.8 mg/dl ) were r and omly assigned to receive seven plasma exchanges ( n = 70 ) or 3000 mg of intravenous methylprednisolone ( n = 67 ) . Both groups received oral cyclophosphamide and oral prednisolone . The primary end point was dialysis independence at 3 mo . Secondary end points included renal and patient survival at 1 yr and severe adverse event rates . At 3 mo , 33 ( 49 % ) of 67 after intravenous methylprednisolone compared with 48 ( 69 % ) or 70 after plasma exchange were alive and independent of dialysis ( 95 % confidence interval for the difference 18 to 35 % ; P = 0.02 ) . As compared with intravenous methylprednisolone , plasma exchange was associated with a reduction in risk for progression to ESRD of 24 % ( 95 % confidence interval 6.1 to 41 % ) , from 43 to 19 % , at 12 mo . Patient survival and severe adverse event rates at 1 yr were 51 ( 76 % ) of 67 and 32 of 67 ( 48 % ) in the intravenous methylprednisolone group and 51 ( 73 % ) of 70 and 35 of ( 50 % ) 70 in the plasma exchange group , respectively . Plasma exchange increased the rate of renal recovery in ANCA-associated systemic vasculitis that presented with renal failure when compared with intravenous methylprednisolone .  Patient survival and severe adverse event rates were similar in both groups In a r and omized study of 26 patients with histologically confirmed rapidly progressive crescentic glomerulonephritis , 12 patients were treated with immunosuppressants alone ( corticosteroids , cyclophosphamide and azathioprine ) while the other 14 patients received not only the identical immunosuppressive treatment but also plasma exchange therapy for four weeks . No statistically significant difference was found between the two groups . After 8 weeks , 73 % and 69 % of the patients in each respective group showed recompensation of renal function ; serum creatinine fell from initially 7.0 and 6.2 mg/dl mean to 2.7 and 2.3 mg/dl mean , and under continued immunosuppression did not rise in the following months . Thus , in non-autoantibody induced rapidly progressive glomerulonephritis , kidney function could be improved substantially by immunosuppressive therapy , but an advantage of supplementary plasma exchange could not be shown BACKGROUND The majority of patients with Wegener 's granulomatosis have disease flares after conventional medications are tapered . There is no consistently safe , effective treatment for the maintenance of remission . METHODS We conducted a r and omized , placebo-controlled trial at eight centers to evaluate etanercept for the maintenance of remission in 180 patients with Wegener 's granulomatosis . The primary outcome was sustained remission , defined as a Birmingham Vasculitis Activity Score for Wegener 's Granulomatosis of 0 for at least six months ( scores can range from 0 to 67 , with higher scores indicating more active disease ) . In addition to etanercept or placebo , patients received st and ard therapy ( glucocorticoids plus cyclophosphamide or methotrexate ) . After remission , st and ard medications were tapered according to the protocol . RESULTS The mean follow-up for the overall cohort was 27 months . Of the 174 patients who could be evaluated , 126 ( 72.4 percent ) had a sustained remission , but only 86 ( 49.4 percent ) remained in remission for the remainder of the trial . There were no significant differences between the etanercept and control groups in the rates of sustained remission ( 69.7 percent vs. 75.3 percent , P=0.39 ) , sustained periods of low-level disease activity ( 86.5 percent vs. 90.6 percent , P=0.32 ) , or the time required to achieve those measures . Disease flares were common in both groups , with 118 flares in the etanercept group ( 23 severe and 95 limited ) and 134 in the control group ( 25 severe and 109 limited ) . There was no significant difference between the etanercept and control groups in the relative risk of disease flares per 100 person-years of follow-up ( 0.89 , P=0.54 ) . During the study , 56.2 percent of patients in the etanercept group and 57.1 percent of those in the control group had at least one severe or life-threatening adverse event or died ( P=0.90 ) . Solid cancers developed in six patients in the etanercept group , as compared with none in the control group ( P=0.01 ) . CONCLUSIONS Etanercept is not effective for the maintenance of remission in patients with Wegener 's granulomatosis .  Durable remissions were achieved in only a minority of the patients , and there was a high rate of treatment-related complications BACKGROUND Current st and ard therapy for Wegener 's granulomatosis and microscopic polyangiitis combines corticosteroids and cyclophosphamide to induce remission , followed by a less toxic immunosuppressant such as azathioprine or methotrexate for maintenance therapy . However , azathioprine and methotrexate have not been compared with regard to safety and efficacy . METHODS In this prospect i ve , open-label , multicenter trial , we r and omly assigned patients with Wegener 's granulomatosis or microscopic polyangiitis who entered remission with intravenous cyclophosphamide and corticosteroids  to receive oral azathioprine ( at a dose of 2.0 mg per kilogram of body weight per day ) or methotrexate ( at a dose of 0.3 mg per kilogram per week , progressively increased to 25 mg per week ) for 12 months . The primary end point was an adverse event requiring discontinuation of the study drug or causing death ; the sample size was calculated on the basis of the primary hypothesis that methotrexate would be less toxic than azathioprine . The secondary end points were severe adverse events and relapse . RESULTS Among 159 eligible patients , 126 ( 79 % ) had a remission , were r and omly assigned to receive a study drug in two groups of 63 patients each , and were followed for a mean ( + /-SD ) period of 29+/-13 months . Adverse events occurred in 29 azathioprine recipients and 35 methotrexate recipients ( P=0.29 ) ; grade 3 or 4 events occurred in 5 patients in the azathioprine group and 11 patients in the methotrexate group ( P=0.11 ) . The primary end point was reached in 7 patients who received azathioprine as compared with 12 patients who received methotrexate ( P=0.21 ) , with a corresponding hazard ratio for methotrexate of 1.65 ( 95 % confidence interval , 0.65 to 4.18 ; P=0.29 ) . There was one death in the methotrexate group . Twenty-three patients who received azathioprine and 21 patients who received methotrexate had a relapse ( P=0.71 ) ; 73 % of these patients had a relapse after discontinuation of the study drug . CONCLUSIONS These results do not support the primary hypothesis that methotrexate is safer than azathioprine . The two agents appear to be similar alternatives for maintenance therapy in patients with Wegener 's granulomatosis and microscopic polyangiitis after initial remission . ( Clinical Trials.gov number , NCT00349674 . OBJECTIVE We performed a single-centre non-blinded clinical trial to compare the clinical efficacies of mycophenolate mofetil ( MMF ) and intermittent cyclophosphamide ( CTX ) pulse therapy as induction treatments in patients with antineutrophil cytoplasmic antibody ( ANCA ) vasculitis ( AAV ) and moderate renal involvement . METHODS Patients with active AAV and serum creatinine < 500 micromol/L received either MMF treatment ( MMF group ) or monthly CTX pulse therapy ( CTX group ) for 6 months . Disease activity was assessed using the Birmingham Vasculitis Activity Score ( BVAS ) . The disease activity , remission rate , renal function and adverse reactions were compared between the two groups . RESULTS A total of 35 patients ( 15 male , 20 female : aged 49.1 + /- 12.2 years ) were enrolled , with 18 in the MMF group and 17 in the CTX group . Of the 35 patients , 28 were MPO-ANCA positive and 2 were PR3-ANCA positive . Four patients were lost to follow-up in the CTX group . At Month 6 , BVAS scores were much lower in the MMF group than in the CTX group ( 0.2 + /- 0.89 versus 2.6 + /- 1.7 , P < 0.05 ) . In the intent-to-treatment analysis , 14 of 18 patients ( 77.8 % ) treated with MMF and 8 of 17 patients receiving CTX ( 47.1 % ) had complete remission with an absolute difference of 30.7 % . Eight of 18 patients ( 44.4 % ) in the MMF group and 2 of 17 patients ( 15.4 % ) in the CTX group recovered renal function . Serum ANCA decreased to normal in 41.7 % of patients in the MMF group and in 16.7 % in the CTX group . Side effects in the MMF group were pneumonia ( 1 ) , herpes zoster ( 1 ) and gastrointestinal symptoms ( 2 ) , and in the CTX group were leukocytopenia ( 1 ) , gastrointestinal distress ( 4 ) and pneumonia ( 1 ) . CONCLUSION Our study suggests that MMF effectively ameliorates disease activity and considerably improves renal function in patients with AAV . Further large-scale multicentre prospect i ve r and omized controlled trials will be needed to confirm these findings OBJECTIVES Results from open-label trials suggest that methotrexate ( MTX ) and leflunomide ( LEF ) are effective for maintenance of remission in Wegener 's granulomatosis ( WG ) , but data from r and omized controlled clinical trails are not yet available . METHODS In this multicentre , prospect i ve r and omized controlled clinical trial , patients with generalized WG were treated either with oral LEF 30 mg/day or oral MTX ( starting with 7.5 mg/week reaching 20 mg/week after 8 weeks ) for 2 yrs following induction of remission with cyclophosphamide . The primary endpoint was the incidence of relapses . Secondary outcome parameters were DEI , BVAS , SF-36 , cANCA-titre , ESR and CRP . RESULTS Fifty-four patients were included in the study , 26 in the LEF-limb , 28 in the MTX-limb . In the LEF-group , six patients relapsed after a median time of 7 months , thereof one major relapse with a new pulmonary manifestation . In the MTX-group , 13 relapses occurred in 6 months , of which seven were major : rapidly progressive glomerulonephritis ( n = 4 ) , pulmonary haemorrhage ( n = 2 ) and one cerebral granuloma . The significantly higher incidence of major relapses in the MTX-limb ( P = 0.037 ) led to premature termination of the study . In the LEF-limb , four patients were withdrawn due to hypertension ( n = 2 ) , peripheral neuropathy ( n = 1 ) and leucopenia ( n = 1 ) . CONCLUSION LEF at a dosage of 30 mg/day appears to be effective in the prevention of major relapses in WG , however , this is associated with an increased frequency of adverse events . Further studies testing other dosing regimens of lower doses of LEF are needed to confirm these promising results in larger patients cohorts Although cyclophosphamide and prednisolone are effective in treating systemic vasculitis , the optimum treatment regimes and duration of treatment are unknown . We r and omized 54 patients aged 15 - 70 years ( median 57.5 years ) with systemic vasculitis ( classical polyarteritis n = 8 , microscopic polyarteritis n = 17 , Wegener 's granulomatosis n = 29 ) to treatment with either pulse cyclophosphamide and prednisolone ( PCYP )  ( n = 24 ) or continuous oral and prednisolone and cyclophosphamide  , with the latter followed 
    Output:
    
Azathioprine has equivalent efficacy as a maintenance agent to cyclophosphamide with fewer episodes of leukopenia .
Mycophenolate mofetil may be equivalent to cyclophosphamide as an induction agent but result ed in a higher relapse rate when tested against Azathioprine in remission maintenance .
Rituximab is an effective remission induction agent .
Methotrexate or Leflunomide are potential choices in remission maintenance therapy .
Oral co-trimoxazole did not reduce relapses significantly in Wegener 's granulomatosis .
Conclusions Plasma exchange is effective in patients with severe ARF secondary to vasculitis .
Pulse cyclophosphamide results in an increased risk of relapse when compared to continuous oral use but a reduced total dose .
Whilst cyclophosphamide is st and ard induction treatment , rituximab and mycophenolate mofetil are also effective .
Azathioprine , methotrexate and leflunomide are effective as maintenance therapy .