ANCA相关小血管炎治疗进展

1、1会计学ANCA相关小血管炎治疗进展相关小血管炎治疗进展2Arthritis Rheum 1994; 37: 187-192ANCA-assiciated vasculitisPauci-immuneImmune-complex血管炎：以血管壁的炎症和纤维素样坏死为病理特血管炎：以血管壁的炎症和纤维素样坏死为病理特征的一组疾病征的一组疾病3Chen M, et al. Postgrad Med J 2005; 81: 723-7Chen M, et al. Kidney Int 2005; 68: 2225-9Chen M, et al. Nephrol Dial Transplant 200

2、7; 22: 139-45Chen M, et al. Medicine 2008; 87: 203-9Chen M, et al. Nephrol Dial Transplant 2010; 25: 2062-545678Jayne DR, et al. J Am Soc Nephrol 2007; 18: 2180-89Jayne DR, et al. J Am Soc Nephrol 2007; 18: 2180-81011Arthritis Rheum 2005, 52: 2461-9 12QJM, 1997; 90, 401-9Arthritis Rheum, 1997; 40: 2

3、187-9813Ann Intern Med. 2009; 150, 670-80 Oral CTX may still be considered in patients with frequent relapses 14Ann Rheum Dis. 2009; 68: 310-7.15RAVE study AAV诱导缓解期对比诱导缓解期对比rituximab 和口服和口服CTX （N=197)，两组均接受激素治疗，两组均接受激素治疗 rituximab: 375 mg/m2 i.v. once weekly for 4 weeks (n=99) CTX: 2 mg/kg/d (n=98)

4、缓解率缓解率: 63/99 vs. 52/98 rituximab 治疗复发较治疗复发较CTX有效有效 Stone JH, et al. N Engl J Med 2010;363:221-32. 1617Jayne et al. N Engl J Med 2003;349:36-44AZA18N Engl J Med 2008; 359, 2790-803MTX19Rheumatology, 2007; 46, 1087-91 20MMF212223Relapses happens despite long-term immunosuppressive therapyEven after p

5、atients progress to ESRD, life-threatening relapses, though not common, still occur.Recently, we reported 5 patients with ESRD who suffered from relapses of AAV, manifested as severe pulmonary hemorrhage. Chen M, et al. Am J Med Sci 2009; 337: 411-4Importance of long-term monitoring of relapse and d

6、isease activity24Chen M, et al. J Rheumatol 2008, 35: 448-50 Relapses are sometimes difficult to be differentiated from secondary infectionsReinstitution of immunosuppressant would be a disaster for those infected. 2526infection 21Active vasculitis8 pulmonary17Cerebral hemorrhage1 peritonitis2Malign

7、ancy 1 GI2ESRD1Cardiovascular events5Unknown 3GI hemorrhage1Total 41Hazard ratio95% CIPAge (increased by 10 years)1.5321.200-1.9560.01eGFR (increased by 10ml/min/1.73m2)0.8330.744-0.9330.01Secondary infections2.7991.586-4.9390.001Chen M, et al. Medicine (Baltimore) 2009; 87: 203-9solid line: with pu

8、lmonary infection, dashed line: without pulmonary infection41/99 deaths occurred in older cohortHazard ratio95% CIPage (increased by 10 years) 2.0561.479-2.8580.01Interstitial fibrosis2.4321.102-5.3670.05Independent predictors of pulmonary infectionSpecial attention should be paid to:Older patientsP

9、atients with underlying pulmonary lesionsPulmonary infections27282930Jayne DR, et al. J Am Soc Nephrol 2007; 18: 2180-831RAVE study AAV诱导缓解期对比诱导缓解期对比rituximab 和口服和口服CTX （N=197)，两组均接受激素治疗，两组均接受激素治疗 rituximab: 375 mg/m2 i.v. once weekly for 4 weeks (n=99) CTX: 2 mg/kg/d (n=98) 缓解率缓解率: 63/99 vs. 52/98

10、rituximab 治疗复发较治疗复发较CTX有效有效 Stone JH, et al. N Engl J Med 2010;363:221-32. 32Relapses happens despite long-term immunosuppressive therapyEven after patients progress to ESRD, life-threatening relapses, though not common, still occur.Recently, we reported 5 patients with ESRD who suffered from relapses of AAV, manifested as severe pulmonary hemorrhage. Chen M, et al. Am J Med Sci 2009; 337: 411-4Importance of long-term monitoring of relapse and disease activity33Chen M, et al. J Rheumatol 20