Predictive factors of chronic kidney disease in primary focal segmental glomerulosclerosis

Abrantes, Marcelo Militão; Cardoso, Luis Sérgio Bahia; Lima, Eleonora Moreira; Silva, José M. Penido; Diniz, José Silvério Santos; Bambirra, Eduardo Alves; Oliveira, Eduardo A.

doi:10.1007/s00467-006-0138-y

Cited by 55 publications

(46 citation statements)

References 52 publications

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“…The initial treatment of primary FSGS, usually with corticosteroids, results in remission of proteinuria only in about 25% of patients. [14][15][16] Prolonged prednisone therapy (mean 9 months) in adults resulted in complete and partial remission rates of 33% and 29%, in observational and uncontrolled trials, respectively. 17 About 60% of patients with persistent nephrotic-range proteinuria progress to end-stage renal disease within 5-10 years.…”

Section: Discussionmentioning

confidence: 99%

Tip lesion variant of primary focal and segmental glomerulosclerosis: clinicopathological analysis of 20 cases

et al. 2015

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The glomerular tip lesion (GTL) is a distinctive histopathologic lesion which is regarded as a variant of focal and segmental glomerulosclerosis (FSGS). The prognostic significance of GTL among other FSGS variants has been disputed. In order to define the clinical features and outcome of GTL, we retrospectively reviewed the presenting clinical features, laboratory and biopsy findings and surveillance in our cohort of GTL, which consisted of 20 adults with native kidneys (mean age 46 years) with follow-up data ranging from 3 to 137 months. At presentation, mean urine protein, serum albumin and cholesterol levels were 5.17 g/d, 2.6 g/dL and 312.9 mg/dL, respectively, and none had renal insufficiency. Microscopic hematuria was detected in five patients. At biopsy, glomerular segmental lesions consisted of GTL without perihilar or collapsing lesions. GTL was observed in a variable proportion of glomeruli from 2.6% to 100%. Mesangial proliferation was seen in nine cases, at a moderate degree in two and mild in the rest. Three biopsies showed mild, two showed moderate interstitial fibrosis/tubular atrophy. Eleven patients received steroids alone and eight received sequential therapy with steroids and a cytotoxic agent. At a mean follow-up of 40.6 months, 17 patients (85%) achieved complete remission of nephrotic syndrome, 15% had partial remission. Four of 17 suffered from recurrences. No patient progressed to end-stage renal disease. Serum albumin at diagnosis was the only predictor of a recurrence (p ¼ 0.037). Microscopic hematuria correlated with incomplete remission (p ¼ 0.045). Our study demonstrates a clearly favorable prognosis in patients with FSGS-GTL variant.

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Section: Discussionmentioning

confidence: 99%

Tip lesion variant of primary focal and segmental glomerulosclerosis: clinicopathological analysis of 20 cases

et al. 2015

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“…While approximately 90% of these patients respond to steroid treatment within 4 weeks, in patients with focal segmental glomerulosclerosis (FSGS) response rates to steroids are as low as 30%. Steroid resistance itself has a poor prognosis for renal survival [2,3]. Furthermore, recent studies discuss a rise in the incidence of steroid resistance [4] and an increasing rate of FSGS in children [5].…”

Section: Introductionmentioning

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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“…[13][14][15] At least in children with FSGS, the presence of hematuria has been linked to a higher risk of progression to chronic kidney disease. 16 This report is a reminder of how AKI may complicate the course of nephrotic syndrome and create a diagnostic challenge. In our case, the combined presence of AKI and hematuria in a patient with collapsing FSGS mimicked an RPGN and prompted aggressive therapy with steroids and cyclophosphamide.…”

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confidence: 93%

Challenges in clinical–pathologic correlations: Acute tubular necrosis in a patient with collapsing focal and segmental glomerulosclerosis mimicking rapidly progressive glomerulonephritis

et al. 2010

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Herein, we report a case of acute kidney injury (AKI) due to diarrhea-induced acute tubular necrosis (ATN) in a patient with nephrotic syndrome secondary to biopsy-proven collapsing focal and segmental glomerulosclerosis (FSGS). The clinical picture mimicked rapidly progressive glomerulonephritis (RPGN) and motivated pulse therapy with methylprednisolone and cyclophosphamide. The case presentation is followed by a brief overview of the epidemiology of AKI in nephrotic syndrome as well as a discussion of its risk factors and potential mechanisms involved.

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Predictive factors of chronic kidney disease in primary focal segmental glomerulosclerosis

Cited by 55 publications

References 52 publications

Tip lesion variant of primary focal and segmental glomerulosclerosis: clinicopathological analysis of 20 cases

Tip lesion variant of primary focal and segmental glomerulosclerosis: clinicopathological analysis of 20 cases

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

Challenges in clinical–pathologic correlations: Acute tubular necrosis in a patient with collapsing focal and segmental glomerulosclerosis mimicking rapidly progressive glomerulonephritis

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