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

Abstract

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.

Pathogenesis of Focal Segmental Glomerulosclerosis

“…Tip variant has a favorable prognosis and good response to therapy in most series [8]. However, contrary reports have also been published [9]. In the cellular variant, at least one glomerulus shows segmental endocapillary hypercellularity, but not in a tip location [1].…”

Histological pattern of focal segmental glomerulosclerosis and its clinical correlation

“…The bias towards females in tip variant was studied by others as well. 6,13 Most of our patients presented with nephrotic syndrome with proteinuria >10 gm/24 hours in 13 (25.4%) patients. Hypertension was seen in 24 (47.05%) patients and renal insufficiency in 31 (60%) patients.…”

“…5,8 Also, there are some studies which did not find any difference in the chronicity of biopsy and the variant of FSGS. 13…”

Acute Myocardial Infarction in a Patient With Focal Segmental Glomerulosclerosis Tip Lesion: Hit With a Double Whammy

“…Traditionally, the tip lesion type of FSGS has been considered to be associated with the best prognosis, with respect to response to treatment and earlier remission rates [7]. However, the lesion may be not as benign as it seems and may be associated with worsening proteinuria, deteriorating renal functions, and difficulty in achieving remission rates [8].…”