Changing incidence of glomerular diseases in adults

Braden, Gregory; Mulhern, Jeffrey G.; O'Shea, Michael; Nash, Shirin; Ucci, Angelo A.; Germain, Michael J.

doi:10.1016/s0272-6386(00)70258-7

Cited by 216 publications

(164 citation statements)

References 17 publications

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“…Nevertheless, the percentage of adult nephrotic patients with FSGS caused by these mutations is currently unknown; these markers are not readily available; and their capacity to predict outcome better than clinical variables such as renal function, proteinuria, and BP has not been demonstrated. Two other factors have been associated with a poor outcome: Patients of black origin and the histologic features of collapsing glomerulopathy (1,(35)(36)(37). Although these factors were not common in our cohort compared with many centers in the United States, their presence did not preclude remission, leaving the issue of to treat or not to treat dependent on the specifics of each case.…”

Section: Discussionmentioning

confidence: 99%

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Focal and Segmental Glomerulosclerosis

Troyanov

Wall

Miller

et al. 2005

Journal of the American Society of Nephrology

242

211

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Focal and segmental glomerulosclerosis (FSGS) is one of the most common primary glomerular diseases to terminate in ESRD.A complete remission (CR) confers an excellent long-term prognosis, but the quantitative benefits of partial remissions (PR) have not been defined. This study evaluated the rate of renal function decline (slope of creatinine clearance) and renal survival in nephrotic FSGS patients with CR, PR, or no remission. It also examined relapse rate from remission and its impact on outcome. Multivariate analysis included clinical and laboratory data at presentation and over follow-up, BP control, the agents used, and immunosuppressive therapy. The study cohort was 281 nephrotic FSGS patients who had a minimum of 12 mo of observation and were identified from the Toronto Glomerulonephritis Registry. Over a median follow-up of 65 mo, 55 experienced a CR, 117 had a PR, and 109 had no remission. A PR was independently predictive of slope and survival from renal failure by multivariate analysis (adjusted time-dependent hazard ratio, 0.48; 95% confidence interval, 0.24 to 0.96; P ‫؍‬ 0.04). Immunosuppression with high-dose prednisone was associated with a higher rate of PR and CR. Relapse from PR was frequent (56%) and associated with a more rapid rate of renal function decline and worse renal survival compared with relapse-free partial remitters. Only female gender and the nadir of proteinuria during remission were associated with a sustained remission. A PR in proteinuria and its maintenance are important therapeutic targets in FSGS, with implications for both slowing progression rate and improving renal survival.J (FSGS) is one of the most common causes of primary glomerular disease that terminates in renal failure (1,2). In most series, its 10-yr survival is in the 40 to 60% range (3-7).Severity of proteinuria at onset and during follow-up have been associated with a poor outcome in several series (3-8).Although there is strong evidence that nephrotic patients who experience a complete remission (CR) have a very favorable prognosis (9 -11), the long-term outcome in adults with only a reduction in proteinuria has seldom been reported (11,12). Despite the lack of evidence of its value as a valid surrogate for improved renal survival in FSGS, it is often reported as a positive finding in clinical studies (11)(12)(13)(14).This study addresses the long-term implications of a partial remission (PR) in nephrotic FSGS patients. It compares the rate of renal function decline, relapse, renal failure, and treatment effects among patients with a PR, CR, and no remission (NR). Materials and MethodsAll FSGS patients from the Toronto Glomerulonephritis Registry were considered for this study. This database began in 1974 and includes all biopsy-proven cases of glomerulonephritis from the Toronto area. Patient information at onset is compiled using a standard form, and registrars perform a periodic prospective assessment of the patients' clinical status, medications, and laboratory results (15). We reviewed all previous m...

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Section: Discussionmentioning

confidence: 99%

“…F ocal and segmental glomerulosclerosis (FSGS) is one of the most common causes of primary glomerular disease that terminates in renal failure (1,2). In most series, its 10-yr survival is in the 40 to 60% range (3)(4)(5)(6)(7).…”

mentioning

confidence: 99%

Focal and Segmental Glomerulosclerosis

Troyanov

Wall

Miller

et al. 2005

Journal of the American Society of Nephrology

242

211

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“…Recent epidemiologic studies have shown that the incidence of FSGS has increased by two to fourfold in the past two decades, although the reasons for this increase are unclear (6,7). There is a racial difference in this syndrome with a two to four times higher prevalence in black compared with white patients (7).…”

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confidence: 99%

“…There is a racial difference in this syndrome with a two to four times higher prevalence in black compared with white patients (7). Overall, it is the single most common primary glomerular disease, responsible for approximately 3% of ESRD in the United States (6,7). At the clinical level, up to 40% adults with FSGS can be expected to respond to a course of intensive immunosuppressive treatment with complete remission of the nephrotic syndrome.…”

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confidence: 99%

Recessive NPHS2 (Podocin) Mutations Are Rare in Adult-Onset Idiopathic Focal Segmental Glomerulosclerosis

Zahirieh

Mei

et al. 2007

Clinical Journal of the American Society of Nephrology

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Recessive NPHS2 (podocin) mutations account for up to approximately 30% of steroid-resistant idiopathic FSGS in children and are associated with a reduced risk for disease recurrence after renal transplantation. R229Q, a missense variant that is present in 3.6% of the white population, has been implicated as a common disease-causing mutation. Given these clinical implications, we examined the role of NPHS2 mutations in a cohort of patients with adult-onset FSGS. We used denaturing HPLC to screen for heterozygous and homozygous gene variants in PCR-amplified DNA fragments that contained all exons and splice junctions of NPHS2. Bidirectional sequencing was performed to define all of the gene variants detected. With the use of the denaturing HPLC in a single-blind pilot study, 40 of 43 known NPHS2 mutations were detected from 22 pediatric patients with FSGS to establish a test sensitivity of 93%. This screen then was applied to 87 adult patients with idiopathic FSGS (15 steroid-sensitive, 63 steroidresistant, and nine familial cases). In this latter cohort, compound heterozygous mutations were detected only in one patient with steroid-sensitive FSGS (R229Q and Q285fsX302) and no homozygous mutations. Overall, R229Q accounted for eight (80%) of ten of the putative mutant alleles that were detected in the study cohort. Contrary to the pediatric experience, recessive NPHS2 mutations are rare in this study population, suggesting that the pathogenesis of FSGS in adults may differ from that in children. These data do not support R229Q as a disease-causing mutation for steroid-resistant FSGS.

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“…Naini EA. et al 3 15 , in their study reported that the incidence was 2%.In this study 5 patients were diagnosed with lupus nephritis where all cases were females. Naini et al 3 have found that Lupus nephritis contributed around 10.6% of cases of adult glomerulonephritides.…”

Section: Discussionmentioning

confidence: 53%