| Theme |
Management of Systemic Diseases with Blood Purification : Aute and Chronic Stage of Diseases |
| Title |
Small-vessel vasculitis (Wegener's granulomatosis, Churg-Strauss syndrome and microscopic polyangiitis) |
| Author |
Hitoshi Yokoyama |
Division of Blood Purification, Kanazawa University School of Medicine |
| Author |
Takashi Wada |
First Department of Internal Medicine, Kanazawa University School of Medicine |
| Author |
Ken-ichi Kobayashi |
First Department of Internal Medicine, Division of Blood Purification, Kanazawa University School of Medicine |
| [ Summary ] |
The mechanisms by which apheresis is of benefit for small-vessel vasculitides remain obscure. The effectiveness of plasma exchange has been reported in some cases with rapidly progressive glomerulonephritis (RPGN) including systemic vasculitides. In five prospective, randomized, controlled trials, there was no statistically significant benefit from plasma exchange for any one group. However, a significant difference was reported in the dialysis dependent group, with RPGN, and systemic involvements, in which 10 out of 11 patients treated by plasma exchange and 3 of 8 control patients recovered renal function by 4th week. In addition, lymphocytapheresis was more effective than methylprednisolone pulse therapy for reduction of glomerular injury due to RPGN, including MPO-ANCA positive patients. In conclusion, the evidence supporting a beneficial role for apheresis in small vessel vasculitides is still low, but dialysis dependent patients are a group who may benefit when there is also severe systemic disease, which is unresponsive to drugs or there is a high risks for complications such as infection. |