Study of the morphologic variants of focal segmental glomerulosclerosis: a Brazilian report

Testagrossa, Leonardo de Abreu; Malheiros, Denise Maria Avancini Costa

doi:10.1590/s1676-24442012000300009

Cited by 7 publications

(13 citation statements)

References 17 publications

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“…Collapsing was the second most common variant in our study, and there is wide variation in the reported prevalence of this variant among different studies, varying from 2% to 36.6% (20). The highest frequency was reported from Brazilian study, where it was the second most common variant after NOS variant (24). It is well known that there is a higher prevalence of collapsing variant in studies which have included African Americans and HIV patients.…”

Section: Discussionmentioning

confidence: 47%

“…The recognition of the histologic variants of FSGS, defined in Columbia classification, can be the first step in this direction in the case of primary FSGS (17,18). Several studies have been published in the literature, emphasizing the wide variation in the prevalence of these variants among different populations (19)(20)(21)(22)(23)(24). It is also well established that certain histologic variants of FSGS are associated with variable presenting clinical features, poor clinical outcome, including rapid deterioration of renal functions and marked proteinuria (20).…”

Section: Discussionmentioning

confidence: 99%

“…There are several studies published in international literature on the characteristics of different histologic variants of FSGS (19)(20)(21)(22)(23)(24). These studies have shown that the variants have different etiologies, pathogenetic mechanisms and diverse clinical behavior in terms of presentation, remission of proteinuria, progression of disease and therapeutic response (19)(20)(21)(22)(23)(24).…”

Section: Introductionmentioning

confidence: 99%

“…These studies have shown that the variants have different etiologies, pathogenetic mechanisms and diverse clinical behavior in terms of presentation, remission of proteinuria, progression of disease and therapeutic response (19)(20)(21)(22)(23)(24). All the studies have reiterated the clinical importance of histologic variants of primary FSGS.…”

Section: Introductionmentioning

confidence: 99%

“…It is also evident from above studies that the relative frequency of the variants of primary FSGS differs in different populations. The frequency of FSGS, NOS variant in published literature varies from 32% to 72.5%, perihilar variant from 4% to 26%, cellular variant from 3% to 25.5%, tip variant from 4.8% to 37% and collapsing variant from 2% to 24% (19)(20)(21)(22)(23)(24). There is no study on the frequency and the clinicopathological presentation of FSGS variants in adult patients from Pakistan.…”

Section: Introductionmentioning

confidence: 99%

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Frequency and clinicopathological characteristics of variants of primary focal segmental glomerulosclerosis in adults presenting with nephrotic syndrome

Shakeel¹,

Mubarak²,

Kazi³

et al. 2013

J Nephropathol

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Background: There is no information on the frequency and clinicopathological presentation of the variants of primary focal segmental glomerulosclerosis (FSGS) in adults presenting with idiopathic nephrotic syndrome (INS) in Pakistan. Objectives: The aim of this study was to determine the frequencies of different histologic variants of primary FSGS with INS at our center and to compare our findings with those published in literature. Patients and Methods: All consecutive adults (≥18 years) with INS, and diagnosis of FSGS on renal biopsies, were included. Their clinicopathological features at the time of presentation were retrieved and compared among the variants. Results: There were 120 (65.2%) males and 64 (34.8%) females. The mean age was 30.62±12.02 years. The mean 24-hr urinary protein excretion was 4.69±2.36 grams. Microscopic hematuria was found in 30 (16.3%) patients. The mean serum creatinine was 1.58±0.87 mg/dL. At presentation, 128 (69.6%) patients were normotensive, while 56 (30.4%) exhibited hypertension. FSGS, not otherwise specified (NOS) was the predominant variant, comprising 76.6% of all; collapsing variant comprised 12%, tip variant, 9.8%, perihilar, 1.1%, and cellular, 0.5%. The mean number of glomeruli involved by segmental scarring was 3.41±2.87 and there was significant difference among the variants (p= 0.001). Arteriolopathy was found in 23.4 % cases and fibrointimal thickening of arteries in 18.5%. Tubular atrophy and interstitial fibrosis (IF/TA) was noted in 93% of cases. There was no significant difference in vasculopathy and IF/TA among the variants. Conclusions: Collapsing variant was the second most common variant following NOS and these findings are different from other regional studies. Implication for health policy/practice/research/medical education:Focal segmental glomerulosclerosis (FSGS) is a heterogeneous lesion with different etiologies, pathogenesis and clinical outcome. Recent classification of FSGS on histological grounds into five variants reflects this heterogeneity of the lesion. A study of these variants in different populations is of utmost importance in understanding the varied etiopathogenetic mechanisms underlying this common lesion in nephrology practice.Please cite this paper as: Shakeel Sh, Mubarak M, Kazi JI, Jafry N, Ahmed E. Frequency and clinicopathological characteristics of variants of primary focal segmental glomerulosclerosis in adults presenting with nephrotic syndrome.

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Section: Discussionmentioning

confidence: 47%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Frequency and clinicopathological characteristics of variants of primary focal segmental glomerulosclerosis in adults presenting with nephrotic syndrome

Shakeel¹,

Mubarak²,

Kazi³

et al. 2013

J Nephropathol

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Idiopathic collapsing focal segmental glomerulosclerosis in an 81-year-old Japanese woman: a case report and review of the literature

et al. 2016

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Focal segmental glomerulosclerosis (FSGS) is classified into five variants, with the collapsing variant being the most rare. Collapsing FSGS is characterized by a black racial predominance and is often associated with human immunodeficiency virus-associated nephropathy. However, the number of idiopathic cases is increasing and the presentation of non-black patients becoming more routine. Our analysis of 15 previous reports investigating FSGS variants shows that the collapsing variant accounts for 10.6 % of FSGS cases and its average age of onset is 32 years old. The current case is one of the oldest cases of idiopathic collapsing FSGS identified, being an 81-year-old Japanese woman. She presented with severe renal insufficiency (serum creatinine 7.9 mg/dL, albumin 1.5 g/dL) and so underwent hemodialysis immediately. Urinalysis demonstrated 3? proteinuria and 3? hematuria and the serological work up was all negative. Renal biopsy showed wrinkling of capillary walls with collapse lumens in every glomerulus, without endothelial tubuloreticular inclusions. Combined treatment with steroids, cyclosporine and lowdensity lipoprotein apheresis increased urine output slightly but she was unable to withdraw from hemodialysis and died 3 months later. This variant is reported to have the highest rate of progression to end-stage renal disease, regardless of the therapeutic intervention. However, there are also examples of cases with partial or complete remission in the literature. Progressive cases, like the current case, seem to be difficult to induce remission in, so it is important to diagnose idiopathic collapsing FSGS at an early stage by performing a renal biopsy, even in elderly patients.

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A Study of Focal and Segmental Glomerulosclerosis according to the Columbia Classification and Its Correlation with the Clinical Outcome

Nuguri¹,

Swain

Padua

et al. 2023

J Lab Physicians

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Introduction Focal and segmental glomerulosclerosis (FSGS) is a leading cause of nephrotic syndrome in both adults and children. The “Columbia classification of FSGS” includes five variants; not otherwise specified (NOS), tip, perihilar, cellular, and collapsing variants that may have different prognostic and therapeutic implications. Materials and Methods This is a retrospective study and was carried out in the Department of Histopathology, Apollo Hospitals, Hyderabad. Of a total of 11,691 kidney biopsies over a 7-year period, from 2006 to 2012, 824 cases were diagnosed as FSGS, of which 610 cases in which detailed clinical findings were available were included in this study. FSGS was then categorized according to the Columbia classification. Results FSGS, NOS was the predominant histomorphological variant. Serum creatinine was significantly high in the collapsing variant, followed by NOS. Follow-up data was available for 103 cases,72.8% had complete remission, 10.6% had partial remission, and in 16.5 % there was no remission. Relapses were observed in 6.7% cases, two patients (1.9%) succumbed, and 4.8% cases progressed to chronic kidney disease. Conclusion This study showed that perihilar variant was less prevalent, with tip and cellular variants being more prevalent in Indian subcontinent compared to Western literature. Collapsing variant was also less common.

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Study of the morphologic variants of focal segmental glomerulosclerosis: a Brazilian report

Cited by 7 publications

References 17 publications

Frequency and clinicopathological characteristics of variants of primary focal segmental glomerulosclerosis in adults presenting with nephrotic syndrome

Frequency and clinicopathological characteristics of variants of primary focal segmental glomerulosclerosis in adults presenting with nephrotic syndrome

Idiopathic collapsing focal segmental glomerulosclerosis in an 81-year-old Japanese woman: a case report and review of the literature

A Study of Focal and Segmental Glomerulosclerosis according to the Columbia Classification and Its Correlation with the Clinical Outcome

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