Can prolonged treatment improve the prognosis in adults with focal segmental glomerulosclerosis?

Ponticelli, Claudio; Villa, Margarita; Banfi, Giovanni; Cesana, Bruno Mario; Pozzi, Claudio; Pani, Antonello; Passerini, P; Farina, Marco; Grassi, Claudio; Baroli, Ambrogio

doi:10.1016/s0272-6386(99)70384-7

Cited by 118 publications

(83 citation statements)

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“…The best predictor of outcome in nephrotic patients with primary FSGS, irrespective of histologic variant, is a remission in proteinuria (1,4,5,16,28). Because spontaneous remissions are rare and the use of conservative management alone rarely leads to remission in nephrotic patients with FSGS, a trial of steroids has been recommended (2)(3)(4)(5).…”

Section: Discussionmentioning

confidence: 99%

“…Because spontaneous remissions are rare and the use of conservative management alone rarely leads to remission in nephrotic patients with FSGS, a trial of steroids has been recommended (2)(3)(4)(5). In cases in which there is concern regarding the use of steroids as initial therapy, such as the obese patient or patients with diabetes, the use of cyclosporine may be a beneficial alternative, but it also has risk (1,29 -31).…”

Section: Discussionmentioning

confidence: 99%

“…In cases in which there is concern regarding the use of steroids as initial therapy, such as the obese patient or patients with diabetes, the use of cyclosporine may be a beneficial alternative, but it also has risk (1,29 -31). Previous attempts to determine which patients are most likely to benefit from a trial of therapy have failed to demonstrate any clinical or histologic features at biopsy that reliably predict response (3,5,16). Recently, Bazzi et al (32) showed that the fractional excretion of IgG, a measure of protein selectivity, is highly predictive of response to steroid and immunosuppressive therapy in their patients with FSGS.…”

Section: Discussionmentioning

confidence: 99%

“…However, it is widely recognized that the prognosis in nephrotic patients with primary FSGS is significantly improved when remission of proteinuria is achieved. Because Ͼ50% of nephrotic adult patients with FSGS respond to an aggressive course of steroids, a trial of therapy has been recommended (1,(3)(4)(5).…”

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Focal Segmental Glomerulosclerosis in Nephrotic Adults

Chun¹,

Korbet²,

Schwartz³

et al. 2004

Journal of the American Society of Nephrology

230

202

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Abstract. The histopathologic diagnosis of primary focal segmental glomerulosclerosis (FSGS) has come to include a number of histologic lesions (variants), but the prognostic significance of these discrete lesions is controversial because published information regarding the presentation, course, and response to treatment is limited. A retrospective analysis was conducted of 87 nephrotic adult patients with biopsy-proven primary FSGS. Patients were categorized on the basis of histologic criteria into those with a classic scar (36 patients), the cellular or collapsing lesion (40 patients), or the tip lesion (11 patients) of FSGS to evaluate differences in presentation, response to therapy, and clinical outcomes. The clinical features at biopsy were similar among the three groups with the exception that patients with the tip lesion were older and patients with the collapsing lesion had more severe proteinuria. Over the course of follow-up, 63% of patients treated attained remission and the response to steroid therapy was similar among the groups (classic scar 53% versus collapsing lesion 64% versus tip lesion 78%; P ϭ 0.45). The overall renal survival was significantly better for patients who entered remission compared with patients who did not enter remission (92% versus 33% at 10 yr; P Ͻ 0.0001). The renal survival at 10 yr for patients who entered remission was similar among the three groups (classic scar 100% versus tip lesion 100% versus collapsing lesion 80%; P ϭ 0.61). In patients who did not enter remission, the renal survival at 10 yr was significantly worse for patients with collapsing lesion and tip lesion (classic scar 49% versus tip lesion 25% versus collapsing lesion 21%; P ϭ 0.002). In conclusion, the prognosis for nephrotic FSGS patients who enter remission is excellent regardless of the histologic lesion. Because the remission rate after treatment is similar among patients with the histologic variants, response to therapy cannot be predicted on the basis of histology alone. Thus, nephrotic patients with primary FSGS should receive a trial of therapy irrespective of the histologic lesion when not contraindicated.Focal segmental glomerulosclerosis (FSGS) is a pattern of injury defined by a segmental scar, which involves some but not all glomeruli. When all of the secondary causes of this pattern of injury are eliminated, the remaining patients receive a diagnosis of primary FSGS. Although patients with primary FSGS may present with any level of proteinuria, clinical concern is greatest for those who present with nephrotic-range proteinuria because without treatment, they have an extremely poor prognosis, progressing to ESRD over the course of 3 to 6 yr (1,2). However, it is widely recognized that the prognosis in nephrotic patients with primary FSGS is significantly improved when remission of proteinuria is achieved. Because Ͼ50% of nephrotic adult patients with FSGS respond to an aggressive course of steroids, a trial of therapy has been recommended (1,3-5).Over the last 20 yr, in addition to the "c...

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

See 2 more Smart Citations

Focal Segmental Glomerulosclerosis in Nephrotic Adults

Chun¹,

Korbet²,

Schwartz³

et al. 2004

Journal of the American Society of Nephrology

230

202

View full text Add to dashboard Cite

show abstract

“…Calcineurin inhibitors (CNIs) may be considered in patients with relative contraindications to high-dose steroids (3). These recommendations are primarily on the basis of small retrospective case series (4,(9)(10)(11)(12)(13)(14)(15), the largest of which comprised 53 patients (10). Although randomized, controlled trials have compared the effectiveness of therapies for steroid-resistant FSGS, data comparing first-line glucocorticoids with other potential first-line therapies are extremely limited (16)(17)(18).…”

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confidence: 99%