PT  - JOURNAL ARTICLE
AU  - Huugen, Dennis
AU  - Cohen Tervaert, Jan Willem
AU  - Heeringa, Peter
TI  - TNF-α Bioactivity-Inhibiting Therapy in ANCA-Associated Vasculitis: Clinical and Experimental Considerations
AID  - 10.2215/CJN.02181205
DP  - 2006 Sep 01
TA  - Clinical Journal of the American Society of Nephrology
PG  - 1100--1107
VI  - 1
IP  - 5
4099  - http://cjasn.asnjournals.org/content/1/5/1100.short
4100  - http://cjasn.asnjournals.org/content/1/5/1100.full
SO  - CLIN J AM SOC NEPHROL2006 Sep 01; 1
AB  - Wegener’s granulomatosis, microscopic polyangiitis, idiopathic necrotizing crescentic glomerulonephritis, and Churg-Strauss syndrome are associated with the presence of ANCA with specificity for myeloperoxidase or proteinase 3. Current therapy consists mainly of corticosteroids and cyclophosphamide, but because this treatment regimen is associated with considerable morbidity, other treatment modalities remain desirable. There is compelling evidence that TNF-α plays an important role in the pathogenesis of ANCA-associated vasculitis. Consequently, inhibition of TNF-α bioactivity potentially results in attenuation of disease. This review discusses whether TNF-α bioactivity-inhibiting drugs are useful in the treatment of ANCA-associated vasculitis. The results of in vitro and in vivo experiments, as well as clinical studies, are evaluated. Although the importance of TNF-α during lesion development is evident, clinical trials that use TNF-α blockers in patients with ANCA-associated vasculitis give mixed results. Importantly, in a large-scale, randomized trial, treatment with etanercept was found not to be effective and resulted in an excess of treatment-related morbidity. It remains to be investigated whether inhibition of TNF-α bioactivity is effective in a subgroup of patients.