Idiopathic collapsing focal segmental glomerulosclerosis: A clinicopathologic study

Valeri, Anthony M.; Barisoni, Laura; Appel, Gerald B.; Seigle, Robert L.; D’Agati, Vivette D.

doi:10.1038/ki.1996.493

Cited by 240 publications

(271 citation statements)

References 36 publications

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“…The FSGS-CT findings support significant differences in demographic features between histologic subtypes. Tip and collapsing variants were more common in teenagers and adults than children (5,6,12,(14)(15)(16)(17)(18)(19). Only 29% of tip patients were children aged 2-12 years at onset of FSGS, whereas 43% were over 18 years.…”

Section: Discussionmentioning

confidence: 96%

“…These variants include the common generic NOS type, the aggressive collapsing type, the abruptly presenting tip type, the early-stage cellular type, and the perihilar type common in secondary adaptive FSGS (13)(14)(15)(16)(17). Several retrospective series have suggested that FSGS histologic subtypes vary in incidence and correlate with remission status and outcome.…”

Section: Discussionmentioning

confidence: 99%

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Association of Histologic Variants in FSGS Clinical Trial with Presenting Features and Outcomes

D’Agati

Alster

Jennette

et al. 2013

Clinical Journal of the American Society of Nephrology

134

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SummaryBackground and objectives FSGS histologic variants have correlated with outcomes in retrospective studies. The FSGS Clinical Trial provided a unique opportunity to study the clinical impact of histologic variants in a well defined prospective cohort with steroid-resistant primary FSGS.Design, setting, participants, & measurements Renal biopsies of 138 FSGS Clinical Trial participants aged 2-38 years enrolled from 2004 to 2008 were analyzed using the Columbia classification by core pathologists. This study assessed the distribution of histologic variants and examined their clinical and biopsy characteristics and relationships to patient outcomes.Results The distribution of histologic variants was 68% (n=94) FSGS not otherwise specified, 12% (n=16) collapsing, 10% (n=14) tip, 7% (n=10) perihilar, and 3% (n=4) cellular. Individuals with not otherwise specified FSGS were more likely to have subnephrotic proteinuria (P=0.01); 33% of teenagers and adults had tip or collapsing variants compared with 10% of children, and subjects with these variants had greater proteinuria and hypoalbuminemia than not otherwise specified patients. Tip variant had the strongest association with white race (86%) and the lowest pathologic injury scores, baseline creatinine, and rate of progression. Collapsing variant had the strongest association with black race (63%, P=0.03) and the highest pathologic injury scores (P=0.003), baseline serum creatinine (P=0.003), and rate of progression. At 3 years, 47% of collapsing, 20% of not otherwise specified, and 7% of tip variant patients reached ESRD (P=0.005).Conclusions This is the first prospective study with protocol-defined immunomodulating therapies confirming poor renal survival in collapsing variant and showing better renal survival in tip variant among steroid-resistant patients.

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Section: Discussionmentioning

confidence: 96%

Section: Discussionmentioning

confidence: 99%

Association of Histologic Variants in FSGS Clinical Trial with Presenting Features and Outcomes

D’Agati

Alster

Jennette

et al. 2013

Clinical Journal of the American Society of Nephrology

134

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“…Anecdotal CG in autoimmune diseases including SLE, SLE-like disease, and mixed connective tissue disease has been previously reported (1,2,(9)(10)(11)(12)(13)(14)(15)(16)(17)(18). The aim of this study is to report an analysis of the clinicopathologic findings and the possible association of renal lesions with CG in the setting of SLE and lupuslike disease and review the literature of the reported cases.…”

Section: Introductionmentioning

confidence: 99%

“…The pathologic appearance is characterized by global or segmental collapse of the glomerular capillary tuft, with wrinkling and retraction of the capillary walls overlaid by epithelial cell proliferation in the Bowman space that is frequently accompanied by tubulointerstitial disease (1)(2)(3). CG is regarded as a podocytopathy, it is predominantly seen in the primary form in patients of African descent, and it has been associated with numerous etiologies, including viral infections (HIV, cytomegalovirus, Parvovirus B19, and hepatitis C virus), drugs (pamidronate and all forms of IFN), and anecdotally, autoimmune diseases (SLE and mixed connective tissue disease) (4,5).…”

Section: Introductionmentioning

confidence: 99%

Collapsing Glomerulopathy in 19 Patients with Systemic Lupus Erythematosus or Lupus-Like Disease

Salvatore

Barisoni

Herzenberg

et al. 2012

Clinical Journal of the American Society of Nephrology

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SummaryBackground and objectives Collapsing glomerulopathy is a podocytopathy with segmental or global wrinkling and collapse of capillary walls and overlying epithelial cell proliferation. Idiopathic collapsing glomerulopathy is a distinct clinicopathologic entity with significant proteinuria, poor response to immunosuppressive therapy, and rapid progression to renal failure. Collapsing glomerulopathy is associated with viral infections, autoimmune disease, and drugs. This work presents the largest group of collapsing glomerulopathy in patients with SLE.Design, setting, participants, & measurements Clinicopathological features were retrospectively studied in 19 patients with SLE (16 patients) or SLE-like (3 patients) disease with collapsing glomerulopathy.Results Initially, 95% of patients had nephrotic syndrome with proteinuria of 3-12 g per 24 hours, creatinine levels of 0.6-9.6 mg/dl, positive lupus serologies, and normal complement levels in 63%. Segmental and/or global collapsing glomerulopathy was seen in 11%-77% of glomeruli. Tubular atrophy with focal microcystic changes and interstitial fibrosis was seen in 35% of patients. Minimal glomerular mesangial deposits were noted in 63% of patients, and extensive foot process effacement was seen in 82% of patients. Initial treatment was with pulse/oral steroids. Follow-up from 13 patients revealed that 7 patients progressed to ESRD at the time of biopsy up to 21 months later, 1 patient returned to normal creatinine (1.1 mg/dl) without proteinuria, and 5 patients had creatinine of 1.2-3.6 mg/dl with proteinuria of 0.37-4 g per 24 hours.Conclusions Collapsing glomerulopathy may be seen in SLE patients presenting with massive proteinuria with or without lupus nephritis, which may have prognostic significance.

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“…Collapsing FSGS was mainly described in patients with HIV-associated nephropathy (HIVAN) (13) but also was recognized as a variant of idiopathic FSGS (11,12). Both idiopathic collapsing FSGS and HIVAN are most commonly seen in young black patients (8 -12,14).…”

mentioning

confidence: 99%

Treatment with IFN-α, -β, or -γ Is Associated with Collapsing Focal Segmental Glomerulosclerosis

Markowitz¹,

Nasr²,

Stokes³

et al. 2010

Clinical Journal of the American Society of Nephrology

191

170

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Background and objectives: Treatment with IFN is rarely associated with nephrotic syndrome and renal biopsy findings of minimal-change disease or FSGS.Design, setting, participants, & measurements: We report 11 cases of collapsing FSGS that developed during treatment with IFN and improved after discontinuation of therapy.Results: The cohort consists of seven women and four men with a mean age of 48.2 yr. Ten of the 11 patients were black. Six patients were receiving IFN-␣ for hepatitis C virus infection (n ‫؍‬ 5) or malignant melanoma (n ‫؍‬ 1), three were receiving IFN-␤ for multiple sclerosis, and two were treated with IFN-␥ for idiopathic pulmonary fibrosis. After a mean and median duration of therapy of 4.0 and 12.6 months, respectively, patients presented with acute renal failure (mean creatinine 3.5 mg/dl) and nephrotic-range proteinuria (mean 24-hour urine protein 9.7 g). Renal biopsy revealed collapsing FSGS with extensive foot process effacement and many endothelial tubuloreticular inclusions. Follow-up was available for 10 patients, all of whom discontinued IFN. At a mean of 23.6 months, nine of 10 patients had improvement in renal function, including one with complete remission and two with partial remission. Among the seven patients with available data, mean proteinuria declined from 9.9 to 3.0 g/d. Four of the seven patients were treated with immunosuppression, and there was no detectable benefit.Conclusions: Collapsing FSGS may occur after treatment with IFN-␣, -␤, or -␥ and is typically accompanied by the ultrastructural finding of endothelial tubuloreticular inclusions. Optimal therapy includes discontinuation of IFN.

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Idiopathic collapsing focal segmental glomerulosclerosis: A clinicopathologic study

Cited by 240 publications

References 36 publications

Association of Histologic Variants in FSGS Clinical Trial with Presenting Features and Outcomes

Association of Histologic Variants in FSGS Clinical Trial with Presenting Features and Outcomes

Collapsing Glomerulopathy in 19 Patients with Systemic Lupus Erythematosus or Lupus-Like Disease

Treatment with IFN-α, -β, or -γ Is Associated with Collapsing Focal Segmental Glomerulosclerosis

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