Corticosteroids for the initial episode of steroid-sensitive nephrotic syndrome

Hodson, Elisabeth M; Hahn, Deirdré; Craig, Jonathan C.

doi:10.1007/s00467-015-3106-6

Cited by 12 publications

(7 citation statements)

References 15 publications

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“…Approximately 80-90% of patients will experience complete remission within the 4 weeks of initiating therapy 169 but some centres also administer three intravenous pulses of methylprednisolone every other day at this point 169 . Patients who do not undergo complete remission (and perhaps those with only a modest partial remission) are categorized as having SRNS and require prompt kidney biopsy and genetic testing 170,171 . Only 30% of children with SSNS maintain remission, 10-20% will have fewer than four relapses and the remaining will have frequent relapses (frequently relapsing nephrotic syndrome, FRNS) or will relapse while on a steroid taper (SDNS).…”

Section: New-onset Nephrotic Syndromementioning

confidence: 99%

Podocytopathies

Kopp

Anders

Suszták

et al. 2020

Nat Rev Dis Primers

307

270

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Podocytopathies are kidney diseases in which direct or indirect podocyte injury drives proteinuria or nephrotic syndrome. In children and young adults, genetic variants in >50 podocyte-expressed genes, syndromal non-podocyte-specific genes and phenocopies with other underlying genetic abnormalities cause podocytopathies associated with steroid-resistant nephrotic syndrome or severe proteinuria. A variety of genetic variants likely contribute to disease development. Among genes with non-Mendelian inheritance, variants in APOL1 have the largest effect size. In addition to genetic variants, environmental triggers such as immune-related, infection-related, toxic and haemodynamic factors and obesity are also important causes of podocyte injury and frequently combine to cause various degrees of proteinuria in children and adults. Typical manifestations on kidney biopsy are minimal change lesions and focal segmental glomerulosclerosis lesions. Standard treatment for primary podocytopathies manifesting with focal segmental glomerulosclerosis lesions includes glucocorticoids and other immunosuppressive drugs; individuals not responding with a resolution of proteinuria have a poor renal prognosis. Renin-angiotensin system antagonists help to control proteinuria and slow the progression of fibrosis. Symptomatic management may include the use of diuretics, statins, infection prophylaxis and anticoagulation. This Primer discusses a shift in paradigm from patient stratification based on kidney biopsy findings towards personalized management based on clinical, morphological and genetic data as well as pathophysiological understanding.

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Section: New-onset Nephrotic Syndromementioning

confidence: 99%

Podocytopathies

Kopp

Anders

Suszták

et al. 2020

Nat Rev Dis Primers

307

270

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“…Response to glucocorticoids is crucial to define the prognosis and to guide further management; based on treatment response, patients are classified as steroid-sensitive or steroid resistant ( Table 3). In children, glucocorticoid therapy is started without histologic confirmation and is usually maintained for 2-3 months, with most patients achieving remission within the end of the first month of treatment (126). Of note, the majority of these children have podocytopathies associated with MCD rather that FSGS lesions, which are more likely to respond to steroids.…”

Section: Treatment Of Primary Fsgsmentioning

confidence: 99%

Autoimmunity in Focal Segmental Glomerulosclerosis: A Long-Standing Yet Elusive Association

Podestà

Ponticelli²

2020

Front. Med.

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Focal segmental glomerulosclerosis (FSGS) is a histological term that describes a pathologic renal entity affecting both adults and children, with a wide array of possible underlying etiologies. Podocyte damage with scarring, the hallmark of this condition, leads to altered permeability of the glomerular barrier, which may result in massive proteinuria and relentless renal function deterioration. A definite cause of focal segmental glomerulosclerosis can be confirmed in a minority of cases, while most forms have been traditionally labeled as primary or idiopathic. Despite this definition, increasing evidence indicates that primary forms are a heterogenous group rather than a single disease entity: several circulating factors that may affect glomerular permeability have been proposed as potential culprits, and both humoral and cellular immunity have been implicated in the pathogenesis of the disease. Consistently, immunosuppressive drugs are considered as the cornerstone of treatment for primary focal segmental glomerulosclerosis, but response to these agents and long-term outcomes are highly variable. In this review we provide a summary of historical and recent advances on the pathogenesis of primary focal segmental glomerulosclerosis, focusing on implications for its differential diagnosis and treatment.

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“…Subgroup analyses according to risk of bias items showed that, in studies at low risk of bias for allocation concealment or blinding, there was no significant difference in the risk for FRNS. However, in studies at high or unclear risk of bias for allocation concealment or blinding, the risk for FRNS was significantly reduced in children treated with longer durations of prednisolone 27. Further high-quality data are anticipated from the PREDNOS trial due for completion this year (ISRCTN16645249).…”

Section: Immunosuppressive Therapymentioning

confidence: 99%

Steroid-sensitive nephrotic syndrome: an evidence-based update of immunosuppressive treatment in children

et al. 2015

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Nephrotic syndrome is one of the most common paediatric glomerular diseases, with an incidence of around two per 100,000 children per year. Corticosteroids are the mainstay of treatment, with 85%-90% of children going into remission with an 8-week course of treatment. Unfortunately, nephrotic syndrome follows a relapsing and remitting course in the majority, with 90% relapsing at least once. About half will progress to frequently relapsing nephrotic syndrome (FRNS) or steroid-dependent nephrotic syndrome (SDNS). Different initial steroid regimens have been evaluated since the first trials in Europe and America in the 1960s. Most trials have been designed to evaluate the optimal duration of the initial therapy, rather than different cumulative doses of corticosteroid, or the management of relapses. Until recently, these data suggested that an initial treatment duration of up to 6 months reduced the number of children developing a relapse, without evidence of increased steroid toxicity. Recently, three large, well-designed randomised control trials were published, which demonstrated no significant reduction in risk of relapse or of developing FRNS by extended treatment compared with 2 or 3 months. While there are few trial data to guide the treatment of individual relapses in steroid-sensitive nephrotic syndrome (SSNS), there is some evidence that a short course of corticosteroid therapy during upper respiratory tract infection may prevent relapse. In patients with FRNS or SDNS who continue to relapse despite low-dose alternate-day steroids a number of non-corticosteroid, steroid-sparing immunosuppressive agents (cyclophosphamide, ciclosporin, tacrolimus, mycophenolate mofetil, levamisole, rituximab) have been shown to reduce the risk of relapse and of FRNS. However, there are limited head-to-head data to inform which agent should be preferred. In this article, we review recent data from randomised trials to update paediatricians on the current evidence supporting interventions in SSNS.

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Corticosteroids for the initial episode of steroid-sensitive nephrotic syndrome

Cited by 12 publications

References 15 publications

Podocytopathies

Podocytopathies

Autoimmunity in Focal Segmental Glomerulosclerosis: A Long-Standing Yet Elusive Association

Steroid-sensitive nephrotic syndrome: an evidence-based update of immunosuppressive treatment in children

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