High-dose oral cyclosporin therapy for recurrent focal segmental glomerulosclerosis in children

Raafat, Reem; Kalia, Alok; Travis, Luther B.; Diven, Steven C.

doi:10.1053/j.ajkd.2004.03.028

Cited by 74 publications

(57 citation statements)

References 30 publications

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“…The use of high-dose CSA in an experimental non-responder protocol was based on the positive experience reported in cases of post-transplantation recurrence of FSGS [38] and reported remission rates of >80% in children with steroid-and CPH-resistant FSGS by CSA doses of up to 20 mg/kg per day [39]. Because case series have demonstrated the effectiveness of highdose MPR treatment, in part in combination with alkylating agents [40][41][42], we offered MPH pulses in an experimental non-responder protocol for patients who failed to remit on CPH.…”

Section: Discussionmentioning

confidence: 99%

Cyclosporin A is superior to cyclophosphamide in children with steroid-resistant nephrotic syndrome—a randomized controlled multicentre trial by the Arbeitsgemeinschaft für Pädiatrische Nephrologie

et al. 2008

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First line immunosuppressive treatment in steroid-resistant nephrotic syndrome in children is still open to discussion. We conducted a controlled multicentre randomized open label trial to test the efficacy and safety of cyclosporin A (CSA) versus cyclophosphamide pulses (CPH) in the initial therapy of children with newly diagnosed primary steroid-resistant nephrotic syndrome and histologically proven minimal change disease, focal segmental glomerulosclerosis or mesangial hypercellularity. Patients in the CSA group (n=15) were initially treated with 150 mg/m 2 CSA orally to achieve trough levels of 120-180 ng/ml, while patients in the CPH group (n=17) received CPH pulses (500 mg/m 2 per month intravenous). All patients were on alternate prednisone therapy. Patients with proteinuria >40 mg/m 2 per hour at 12 weeks of therapy were allocated to a nonresponder protocol with high-dose CSA therapy or methylprednisolone pulses. At week 12, nine of the 15 (60%) CSA patients showed at least partial remission, evidences by a reduction of proteinuria <40 mg/h per m 2 . In contrast, three of the 17 (17%) CPH patients responded (p<0.05, intention-totreat). Given these results, the study was stopped, in accordance with the protocol. After 24 weeks, complete remission was reached by two of the 15 (13%) CSA and one of the 17 (5%) CPH patients (p=n.s.). Partial remission was achieved by seven of the 15 (46%) CSA and two of the 15 (11%) CPH patients (p<0.05). Five patients in the CSA group and 14 patients in the CPH group were withdrawn from the study, most of them during the non-responder protocol. The number of adverse events was comparable between both groups. We conclude that CSA is more effective than CPH in inducing at least partial remission in steroid-resistant nephrotic syndrome in children. Conflict of interest statement

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Section: Discussionmentioning

confidence: 99%

Cyclosporin A is superior to cyclophosphamide in children with steroid-resistant nephrotic syndrome—a randomized controlled multicentre trial by the Arbeitsgemeinschaft für Pädiatrische Nephrologie

et al. 2008

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“…Overall, limited evidence has supported the administration of high dose CyA to achieve remission of FSGS recurrence with a persistent effect [45,46] . Salomon et al [45] reported a remission of recurrent proteinuria in 14/17 (82%) of children following administration of intravenous CyA (mean period of 21 d; range of 250-350 ng/mL); after 4 years, 11/17 (64%) patients vided major advancements in our knowledge of the pathophysiology of FSGS, the treatment remains largely empirical.…”

Section: Cyclosporinementioning

confidence: 99%

“…Complete remission in 14/17 (82%); partial remission in 2/17 (12%) Raafat et al [46] Progressive up-titration of CyA oral doses until proteinuria reduction/serum creatinine elevation (CyA doses from 6 to 25 mg/kg per day) [53] CYC (1-2 mg/kg per day, adjusted for white blood cell count) for 8-12 wk 3 patients (< 18 yr) Complete remission in 2/3; partial remission in 1/3…”

Section: Plasma Exchangementioning

confidence: 99%

Update on the treatment of focal segmental glomerulosclerosis in renal transplantation

Messina¹,

Gallo²,

Mella³

et al. 2016

WJT

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Focal segmental glomerulosclerosis (FSGS) represents one of the most severe glomerular diseases, with frequent progression to end-stage renal disease and a high rate of recurrence in renal allografts (30%-50%). Recurrent FSGS portends a negative outcome, with the hazard ratio of graft failure being two-fold higher then that of other glomerulonephritis. Two patterns of clinical presentations are observed: Early recurrence, which is characterized by massive proteinuria within hours to days after implantation of the renal graft, and late recurrence, which occurs several months or years after the transplantation. Many clinical conditions have been recognized as risk factors for recurrence, including younger age, rapid progression of the disease to end-stage renal disease on native kidneys, and loss of previous renal allografts due to recurrence. However, much less is known about the incidence and risk factors of the so-called "de novo " type of FSGS, for which sufferers are transplanted patients without disease on native kidneys; but, rapid development of allograft failure is frequently observed. Management of both forms is challenging, and none of the approaches proposed to date have been demonstrated as consistently beneficial or effective. In the present review we report an update on the available therapeutic strategies for FSGS in renal transplantation within the context of a critical overview of the current literature.

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“…In report of Salomon et al in 14 out of 17 children treated with CsA (3mg/kg/day intravenously) for 3 weeks followed by an oral dosage to maintain trough levels between 200 and 300 ng/ml remission was achieved within 28 days (61). In other report the highdose oral CsA (initially from 6 mg/kg/day in two divided doses) was necessary for inducting remission with complete and partial remission in 81% of treated patients (62). High dose CsA has also been used in connection with PP, but also the success was variable (38; 41; 62).…”

Section: Immunosuppressionmentioning

confidence: 99%

Focal Segmental Glomerulosclerosis (FSGS) Recurrence in Kidney Allograft Recipients

Mazanowska¹,

Kamińska²

2011

After the Kidney Transplant - The Patients and Their Allograft

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High-dose oral cyclosporin therapy for recurrent focal segmental glomerulosclerosis in children

Cited by 74 publications

References 30 publications

Cyclosporin A is superior to cyclophosphamide in children with steroid-resistant nephrotic syndrome—a randomized controlled multicentre trial by the Arbeitsgemeinschaft für Pädiatrische Nephrologie

Cyclosporin A is superior to cyclophosphamide in children with steroid-resistant nephrotic syndrome—a randomized controlled multicentre trial by the Arbeitsgemeinschaft für Pädiatrische Nephrologie

Update on the treatment of focal segmental glomerulosclerosis in renal transplantation

Focal Segmental Glomerulosclerosis (FSGS) Recurrence in Kidney Allograft Recipients

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