Plasma exchange in focal necrotizing glomerulonephritis without anti-GBM antibodies

Abstract

To determine whether plasma exchange was of additional benefit in patients treated with oral immunosuppressive drugs for focal necrotizing glomerulonephritis (without anti-GBM antibodies), we performed a randomized controlled trial with stratification for renal function on entry. Forty-eight cases were analyzed, 25 in the treatment group (plasma exchange, prednisolone, cyclophosphamide and azathioprine) and 23 in the control group (drug therapy only). There was no difference in outcome in patients presenting with serum creatinine less than 500 mumol/liter (N = 17), or greater than 500 mumol/liter but not on dialysis (N = 12), all but one of whom had improved by four weeks. However, patients who were initially dialysis-dependent (N = 19) were more likely to have recovered renal function (P = 0.041) if treated with plasma exchange as well as drugs (10 of 11) rather than with drugs alone (3 of 8). Long-term follow-up showed that improvement in renal function was generally maintained. The results of this trial confirm that focal necrotizing glomerulonephritis related to systemic vasculitis responds well to immunosuppressive drugs when treatment is started early, and suggest that plasma exchange is of additional benefit in dialysis-dependent cases.

Advances in apheresis therapy for glomerular diseases

“…The effectiveness of PE has been reported in some patients with rapidly progressive glomerulonephritis (RPGN), in which ANCA-associated vasculitis is the most common cause. In 6 randomized controlled trials [17][18][19][20][21][22], there was no statistically significant benefit from plasma exchange in a whole group, however. The significant difference was reported in the dialysis-dependent group with RPGN and systemic involvement, in which 10 out of 11 patients treated by PE and 3 of 8 control patients recovered renal function by 4 weeks in previous studies.…”

An Unusual Case of Hematuria

“…In addition, our patient's serum creatinine rapidly worsened during her hospitalization, with a serum creatinine level Ͻ500 moles/liter (5.7 mg/dl), suggestive of severe and rapidly progressive renal vasculitis, immediately life-threatening disease, and poor renal outcome at a 1-year interval (30); therefore, she was treated with a course of plasma exchange (7 sessions in 2 weeks) in conjunction with the prednisone and oral CYC. Plasma exchange has been shown to be beneficial in patients with segmental necrotizing glomerulonephritis and severe renal disease (36,37). The randomized MEPEX trial enrolled 137 patients with a new diagnosis of pauci-immune glomerulonephritis and the results supported the findings that plasma exchange was more effective than methylprednisolone pulses in renal function recovery (33).…”

Wegener Granulomatosis: Case Report and Brief Literature Review

“…5,24 In those with severe disease at presentation, pulmonary hemorrhage, or worsening disease despite immunosuppressive therapy plasmapheresis may be indicated. 27,28 There is not much clinical data to address the long-term outcome in patients who undergo plasmapheresis for WG. 29,30 In our case, the patient began therapy with monthly intravenous cyclophosphamide pulse therapy.…”