Background: Intravenous cyclophosphamide is considered the gold standard in lupus nephritis (LN), a major complication in Systemic lupus erythematosus (SLE), even if emerging evidences show that mycophenolate mofetil could more effective with a more favourable safety profile.¹ Objectives: Our work reports the results of a retrospective study of 44 patients (pts) with LN followed at IRCCS San Raffaele Hospital-Milan, from September 1992 to May 2008, and compares them with 4 multicentric trials. Methods: Forty-four pts (39F, 5M, mean age 34.25 years) with SLE and LN were treated for a mean of 76.6 months. All 44 pts fulfilled ARC criteria for SLE. Renal biopsy was performed in 40 pts: 9 were class III WHO, 27 class IV, 4 class V. Forty pts were treated with monthly pulses of cyclophosphamide (CYC) (750-1000 mg/m2) for a mean of 7 months. In pts with persistent proteinuria after the first 6 CYC pulses, cyclosporin A ( CyA 3mg/Kg die) for 3-8 months was added. According to increasing evidences of MMF efficacy, to CYC toxicity and to nephritis residual activity, monthly CYC pulses were followed by additional CYC pulses every 3 months (30 pts) or by mycophenolate mofetil (MMF) (2 gr die) (6 pts). As maintainance therapy, 19 pts were treated with azathioprine (2 mg/Kg die) for 2 more years. At baseline, 4 pts in which CYC was contraindicated, started immediately with MMF. Results: Therapeutic response was evaluated according to Boumpas and Balow criteria: 39 pts achieved complete remission (CR) after a mean of 16.5 months; 3 pts underwent partial remission and 2 are actually on dialysis after disease progression. In 8 pts relapse occurred after a mean of 34.4 months. One pt died for legionellosis and 1 for cerebral haemorrhage. Infectious complications occurred in 10/44 pts: 2 had pneumonia, 1 measles, 6 herpes zoster and 1 cytomegalovirus infection. During CYC treatment 1 pt developed hemorrhagic cystitis, 1 vulvar squamocellular carcinoma and 5/31 fertile females had amenorrhea. Conclusions: CR was observed in 84% of pts compared to Illei’s 50% and to Mok’s 54%. We observed 2/44 cases of terminal nephritis compared to Austin’s 1/20, Mok’s 10/189 and Houssiau’s 4/85. Disease relapse occurred in 18% of our pts, compared to Illei’s 45.8% and to Mok’s 27.4%. We observed a single case of lethal infection and of death not correlated with therapy, compared to Austin’s 3/20. Our results suggest that an individualized therapeutic approach tailored on individual patient response can improve the outcome of LN compared to standardized protocols.
Individualized therapy provides a better outcome in patients with proliferative lupus nephritis: follow up of a cohort of Italian patients
DELLA TORRE E;
2009-01-01
Abstract
Background: Intravenous cyclophosphamide is considered the gold standard in lupus nephritis (LN), a major complication in Systemic lupus erythematosus (SLE), even if emerging evidences show that mycophenolate mofetil could more effective with a more favourable safety profile.¹ Objectives: Our work reports the results of a retrospective study of 44 patients (pts) with LN followed at IRCCS San Raffaele Hospital-Milan, from September 1992 to May 2008, and compares them with 4 multicentric trials. Methods: Forty-four pts (39F, 5M, mean age 34.25 years) with SLE and LN were treated for a mean of 76.6 months. All 44 pts fulfilled ARC criteria for SLE. Renal biopsy was performed in 40 pts: 9 were class III WHO, 27 class IV, 4 class V. Forty pts were treated with monthly pulses of cyclophosphamide (CYC) (750-1000 mg/m2) for a mean of 7 months. In pts with persistent proteinuria after the first 6 CYC pulses, cyclosporin A ( CyA 3mg/Kg die) for 3-8 months was added. According to increasing evidences of MMF efficacy, to CYC toxicity and to nephritis residual activity, monthly CYC pulses were followed by additional CYC pulses every 3 months (30 pts) or by mycophenolate mofetil (MMF) (2 gr die) (6 pts). As maintainance therapy, 19 pts were treated with azathioprine (2 mg/Kg die) for 2 more years. At baseline, 4 pts in which CYC was contraindicated, started immediately with MMF. Results: Therapeutic response was evaluated according to Boumpas and Balow criteria: 39 pts achieved complete remission (CR) after a mean of 16.5 months; 3 pts underwent partial remission and 2 are actually on dialysis after disease progression. In 8 pts relapse occurred after a mean of 34.4 months. One pt died for legionellosis and 1 for cerebral haemorrhage. Infectious complications occurred in 10/44 pts: 2 had pneumonia, 1 measles, 6 herpes zoster and 1 cytomegalovirus infection. During CYC treatment 1 pt developed hemorrhagic cystitis, 1 vulvar squamocellular carcinoma and 5/31 fertile females had amenorrhea. Conclusions: CR was observed in 84% of pts compared to Illei’s 50% and to Mok’s 54%. We observed 2/44 cases of terminal nephritis compared to Austin’s 1/20, Mok’s 10/189 and Houssiau’s 4/85. Disease relapse occurred in 18% of our pts, compared to Illei’s 45.8% and to Mok’s 27.4%. We observed a single case of lethal infection and of death not correlated with therapy, compared to Austin’s 3/20. Our results suggest that an individualized therapeutic approach tailored on individual patient response can improve the outcome of LN compared to standardized protocols.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
