Initial Steroid Sensitivity in Children with Steroid-Resistant Nephrotic Syndrome Predicts Post-Transplant Recurrence

Ding, Wen Yi; Koziell, Ania; McCarthy, Hugh; Bierżyńska, Agnieszka; Bhagavatula, Murali; Dudley, Jan; Inward, Carol; Coward, Richard J M; Tizard, Jane; Reid, Christopher; Antignac, Corinne; Boyer, Olivia; Saleem, Moin A.

doi:10.1681/asn.2013080852

Cited by 98 publications

(101 citation statements)

References 32 publications

(39 reference statements)

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“…Thus, in a recent study, patients with FSGS who had initial steroid responsiveness that evolved to secondary steroid resistance had a high risk of recurrent disease. In contrast, patients with initial steroid resistance were more likely to have a genetic cause of FSGS and a significantly lower likelihood of developing recurrence posttransplant [26].…”

Section: Pathogenesismentioning

confidence: 86%

Recurrent focal segmental glomerulosclerosis after kidney transplantation

2015

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Focal segmental glomerulosclerosis (FSGS) is an important cause of glomerular disease in children and adolescents and nearly 50 % of affected patients will progress to endstage kidney disease over a 5 to 10-year period. Unfortunately, there is no established treatment for disease in the native kidney. Moreover, up to 55 % of patients develop recurrent disease after receiving a kidney transplant, with a substantially higher risk in patients who have already experienced recurrent disease in a prior transplant. A number of clinical and laboratory factors have been identified as risk factors for this complication. In addition, new investigations into podocyte biology and circulating permeability factors have shed light on the cause of recurrent the disease. While a number of novel therapeutic agents have been applied in the management of this problem, there still is no proven treatment. In this review, we summarize recent advances in the epidemiology, pathophysiology, and treatment of recurrent FSGS in pediatric patients who have received a kidney transplant.

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Section: Pathogenesismentioning

confidence: 86%

Recurrent focal segmental glomerulosclerosis after kidney transplantation

2015

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“…18 This suggests that targeting selected patients earlier in their disease for intense immunosuppression may be beneficial, and a trial of for example pretransplant plasma exchange in targeted patients could be designed.…”

Section: Discussionmentioning

confidence: 99%

Genomic and clinical profiling of a national nephrotic syndrome cohort advocates a precision medicine approach to disease management

Bierżyńska

McCarthy

Soderquest

et al. 2017

Kidney International

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215

241

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“…Rarely, children with initially steroid-sensitive disease will develop secondary steroid resistance. This clinical feature is highly predictive of post-transplant recurrence (78). In adults, relapses are frequent, occurring in about 56%-76% of patients (12,79).…”

Section: Outcomementioning

confidence: 96%

Minimal Change Disease

Vivarelli

Massella

Ruggiero

et al. 2016

CJASN

368

348

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Minimal change disease (MCD) is a major cause of idiopathic nephrotic syndrome (NS), characterized by intense proteinuria leading to edema and intravascular volume depletion. In adults, it accounts for approximately 15% of patients with idiopathic NS, reaching a much higher percentage at younger ages, up to 70%-90% in children >1 year of age. In the pediatric setting, a renal biopsy is usually not performed if presentation is typical and the patient responds to therapy with oral prednisone at conventional doses. Therefore, in this setting steroid-sensitive NS can be considered synonymous with MCD. The pathologic hallmark of disease is absence of visible alterations by light microscopy and effacement of foot processes by electron microscopy. Although the cause is unknown and it is likely that different subgroups of disease recognize a different pathogenesis, immunologic dysregulation and modifications of the podocyte are thought to synergize in altering the integrity of the glomerular basement membrane and therefore determining proteinuria. The mainstay of therapy is prednisone, but steroid-sensitive forms frequently relapse and this leads to a percentage of patients requiring second-line steroid-sparing immunosuppression. The outcome is variable, but forms of MCD that respond to steroids usually do not lead to chronic renal damage, whereas forms that are unresponsive to steroids may subsequently reveal themselves as FSGS. However, in a substantial number of patients the disease is recurrent and requires long-term immunosuppression, with significant morbidity because of side effects. Recent therapeutic advances, such as the use of anti-CD20 antibodies, have provided long-term remission off-therapy and suggest new hypotheses for disease pathogenesis.

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Initial Steroid Sensitivity in Children with Steroid-Resistant Nephrotic Syndrome Predicts Post-Transplant Recurrence

Cited by 98 publications

References 32 publications

Recurrent focal segmental glomerulosclerosis after kidney transplantation

Recurrent focal segmental glomerulosclerosis after kidney transplantation

Genomic and clinical profiling of a national nephrotic syndrome cohort advocates a precision medicine approach to disease management

Minimal Change Disease

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