Tip variant of focal segmental glomerulosclerosis: outcome and comparison to 'not otherwise specified' variant

Arias, Luis Fernando; Franco-Alzate, Catalina; Rojas, Shirley L.

doi:10.1093/ndt/gfq668

Cited by 12 publications

(12 citation statements)

References 22 publications

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“…9,12, 23 Arias et al, have observed progression to chronic kidney disease (CKD) and even to end-stage renal disease (ESRD) in 19.2% of patients with tip lesion and they proposed that tip variant should not be considered as a prognostically favorable disease; rather, it could be an early morphologic insignia of severe glomerular disease. 26,27 Similar observations were made by Howie et al who have proposed that glomerular tip lesion may progress to NOS lesion and it should not be considered as a benign disease. 28 Conclusively, glomerular tip lesion is a distinct morphologic pattern of FSGS which usually exhibits more favorable clinical course in terms of remarkable response to steroid therapy but in long-term follow up studies, tip lesion may progress to CKD and even to ESRD.…”

Section: Tip Variantsupporting

confidence: 66%

Histologic Variants of FSGS: An Epidemiological and Pathological Perspective.

Shakeel

Rashid

Mubarak³

2021

Pak J Kidney Dis

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Focal segmental glomerulosclerosis (FSGS) is a conglomerate of glomerular pathological lesions unified by the segmental distribution of proliferative, sclerosing or collapsing lesions in less than 50% of glomeruli (i.e., focal distribution) in the early stages of the process and can only be diagnosed on renal biopsy. Epidemiologically, FSGS has acquired the top position among the causes of nephrotic syndrome (NS) in adults in many parts of the world and has emerged as the main cause of chronic kidney disease (CKD) and end-stage renal disease (ESRD) worldwide. Numerous approaches have been used to classify this lesion, but all have merits and demerits and no one system is entirely satisfactory. One of the popular schemes for the classification is based on morphological features and distribution of the lesions in the glomeruli, the so-called Columbia classification of FSGS. In this review, we briefly summarize the relevant epidemiology and pathology of FSGS variants in the light of our own experience with the use of this classification.

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Section: Tip Variantsupporting

confidence: 66%

Histologic Variants of FSGS: An Epidemiological and Pathological Perspective.

Shakeel

Rashid

Mubarak³

2021

Pak J Kidney Dis

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“…A number of studies have reported that tip variant has clinical features similar to that of minimal change disease and its responsiveness to corticosteroids is favorable; thus, tip variant is considered to have good prognosis [ 10 , 20 ]. In line with this, in the present study, tip variant was significantly associated with achieving CR or PR.…”

Section: Discussionmentioning

confidence: 99%

Clinical features and outcomes of focal segmental glomerulosclerosis pathologic variants in Korean adult patients

et al. 2014

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BackgroundMany studies have shown that clinical characteristics and outcomes differ depending on pathologic variants of focal segmental glomerulosclerosis (FSGS). However, these are not well defined in Asian populations.MethodsThis retrospective study evaluated clinical features and outcomes of pathologic FSGS variants in 111 adult patients between January 2004 and December 2012. Primary outcome was the composite of doubling of baseline serum creatinine concentrations (D-SCr) or onset of end-stage renal disease (ESRD). Secondary outcome included complete (CR) or partial remission (PR).ResultsThere were 70 (63.1%), 20 (18.0%), 17 (15.3%), 3 (2.7%), and 1 (0.9%) patients with not-otherwise specified (NOS), tip, perihilar, cellular, and collapsing variants, respectively. At presentation, nephrotic-range proteinuria occurred more commonly in tip lesion than in other variants. The overall 5-year renal survival rate was 76.8%. During a median follow-up of 34.5 months, only 1 (5.0%) patient with a tip lesion reached the composite end point compared to 2 (11.8%) and 12 (17.1%) patients in perihilar and NOS variants, but this difference was not statistically significant in an adjusted Cox model. However, tip lesion was associated with a significantly increased probability of achieving CR (P = 0.044).ConclusionSimilar to other populations, Korean adult patients with FSGS have distinct clinical features with the exception of a rare frequency of cellular and collapsing variants. Although pathologic variants were not associated with overall outcome, the tip variant exhibited favorable outcome in terms of achieving remission. Further studies are required to delineate long-term outcome and response to treatment of the pathologic variants.

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“…In a study by Arias et al 23 though 14.9% of patients belonged to the tip variant and had a significant less evidence of chronic damage on histopathology (GS, IFTA, AH), the overall prognosis was not favorable with 28% of patients progressing to CKD and 16% progressing to ESRD.…”

Section: Discussionmentioning

confidence: 94%

Tip variant of focal segmental glomerulosclerosis: is it truly a benign variant?

et al. 2015

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Background: Even though frequently described as a benign entity, the outcomes of the tip variant of focal segmental glomerulosclerosis (FSGS) have proven to be unclear. Methods: This retrospective study includes a cohort of tip variant cases who presented to us from 2009 to 2012 and the analysis of their presenting clinical, histopathological features and treatment outcomes in comparison to the not otherwise specified (NOS) variants from our center in East India. Results: Of the 224 biopsies of primary FSGS, 30 cases were the tip variant (13.39%). The mean age of presentation was around 29 years, with 57% being males. A nephrotic presentation was seen in 87% of cases, with 20% showing a presentation at518 years of age for the first time. Global sclerosis, interstitial fibrosis, tubular atrophy and arteriolar hyalinosis were seen more commonly in the NOS variant. Twenty five patients of tip variant received steroid therapy and eight received alternative immunosuppression. Around 87% of the tip variant cases achieved some form of remission in proteinuria and 13.3% had a doubling of creatinine at a median follow-up of 2 years in comparison to NOS group in which 80% achieved some form of remission and 20% had a doubling of creatinine. Conclusion: Though the histopathological features and treatment responsiveness of the tip variant appear to be better than the NOS variety, the prognostic outcome does not seem to be as favorable as implicated previously with an important percentage of patients showing progressive worsening of renal function within a relatively short time span (2 years) in our cohort.

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Tip variant of focal segmental glomerulosclerosis: outcome and comparison to 'not otherwise specified' variant

Cited by 12 publications

References 22 publications

Histologic Variants of FSGS: An Epidemiological and Pathological Perspective.

Histologic Variants of FSGS: An Epidemiological and Pathological Perspective.

Clinical features and outcomes of focal segmental glomerulosclerosis pathologic variants in Korean adult patients

Tip variant of focal segmental glomerulosclerosis: is it truly a benign variant?

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