The glomerular tip lesion: A previously undescribed type of segmental glomerular abnormality

Howie, Alexander J.; Brewer, D. B.

doi:10.1002/path.1711420308

Cited by 102 publications

(43 citation statements)

References 11 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…This variant is marked by a segmental lesion at the tubular pole, adjacent to the origin of the proximal tubule. The biopsy specimen in this case fits the original description of the GTL, which reported that the remaining glomeruli show no alterations except for diffuse podocyte effacement on electron microscopy, akin to the findings of minimal-change disease (5). The Columbia classification of FSGS uses less restrictive criteria and defines the tip variant as having the presence of at least one tip lesion in the absence of collapsing lesions or perihilar sclerosis (6).…”

Section: Question 2asupporting

confidence: 71%

American Society of Nephrology Quiz and Questionnaire 2015: Glomerular Diseases

Bomback

Perazella

Choi

2016

CJASN

View full text Add to dashboard Cite

The Nephrology Quiz and Questionnaire remains an extremely popular session for attendees of the annual Kidney Week meeting of the American Society of Nephrology. Once again, the conference hall was overflowing with audience members and eager quiz participants. Topics covered by the expert discussants included electrolyte and acid-base disorders, glomerular disease, ESRD/dialysis, and kidney transplantation. Complex cases representing each of these categories, along with single-best-answer questions, were prepared and submitted by the panel of experts. Before the meeting, training program directors of United States nephrology fellowship programs and nephrology fellows answered the questions through an Internet-based questionnaire. During the live session, members of the audience tested their knowledge and judgment on a series of case-oriented questions prepared and discussed by the experts. They compared their answers in real time using their cell phones with a special app with the answers of the nephrology fellows and training program directors. The correct and incorrect answers were then discussed after the results of the questionnaire were displayed. As always, the audience, lecturers, and moderators enjoyed this educational session. This article recapitulates the session and reproduces its educational value for Clinical Journal of the American Society of Nephrology readers. Enjoy the clinical cases and expert discussions.

show abstract

Section: Question 2asupporting

confidence: 71%

American Society of Nephrology Quiz and Questionnaire 2015: Glomerular Diseases

Bomback

Perazella

Choi

2016

CJASN

View full text Add to dashboard Cite

show abstract

“…18 Several reports in the past have shown an excellent response to steroid therapy in the tip variant with outcomes similar to the minimal change disease rather than FSGS. [12][13][14][15]24 However, it has also been shown that the overall behavior and ultimate outcome of this disease are more like FSGS as was seen in our study also. 9,[20][21][22] The incidence of initial steroid responsiveness was higher in the tip variant with 72% of patients achieving complete remission after steroid therapy.…”

Section: Discussionsupporting

confidence: 73%

“…7 It has been postulated that this lesion is the response of the glomeruli to heavy proteinuria and cases with this subtype of FSGS with no other lesion on histopathology may actually represent a form of minimal change disease (MCN). [12][13][14] However, there have been various studies which have shown that despite the histopathological similarities to the benign MCN the clinical course of the disease is similar to that of primary FSGS. 9,15,16 Hence with the aim to study the clinical and histopathological features and the natural history and response to therapy of this variant of FSGS, we retrospectively reviewed our cases of primary FSGS and the tip lesion variant and compared the clinical and biochemical parameters as well as outcome of these patients with those of a relatively common histological variant of NOS.…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2015

View full text Add to dashboard Cite

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.

show abstract

“…Initial reports in nephrotic patients with the tip lesion suggested an excellent response to steroids and favorable course similar to that of minimal-change disease rather than FSGS (14,21,23,24). Subsequent reports found that the response and course in patients with the tip lesion was similar to that of patients with FSGS and thus questioned the clinical significance of this feature (6,10,25,26).…”

Section: Discussionmentioning

confidence: 99%

Focal Segmental Glomerulosclerosis in Nephrotic Adults

Chun¹,

Korbet²,

Schwartz³

et al. 2004

Journal of the American Society of Nephrology

228

202

View full text Add to dashboard Cite

Abstract. The histopathologic diagnosis of primary focal segmental glomerulosclerosis (FSGS) has come to include a number of histologic lesions (variants), but the prognostic significance of these discrete lesions is controversial because published information regarding the presentation, course, and response to treatment is limited. A retrospective analysis was conducted of 87 nephrotic adult patients with biopsy-proven primary FSGS. Patients were categorized on the basis of histologic criteria into those with a classic scar (36 patients), the cellular or collapsing lesion (40 patients), or the tip lesion (11 patients) of FSGS to evaluate differences in presentation, response to therapy, and clinical outcomes. The clinical features at biopsy were similar among the three groups with the exception that patients with the tip lesion were older and patients with the collapsing lesion had more severe proteinuria. Over the course of follow-up, 63% of patients treated attained remission and the response to steroid therapy was similar among the groups (classic scar 53% versus collapsing lesion 64% versus tip lesion 78%; P ϭ 0.45). The overall renal survival was significantly better for patients who entered remission compared with patients who did not enter remission (92% versus 33% at 10 yr; P Ͻ 0.0001). The renal survival at 10 yr for patients who entered remission was similar among the three groups (classic scar 100% versus tip lesion 100% versus collapsing lesion 80%; P ϭ 0.61). In patients who did not enter remission, the renal survival at 10 yr was significantly worse for patients with collapsing lesion and tip lesion (classic scar 49% versus tip lesion 25% versus collapsing lesion 21%; P ϭ 0.002). In conclusion, the prognosis for nephrotic FSGS patients who enter remission is excellent regardless of the histologic lesion. Because the remission rate after treatment is similar among patients with the histologic variants, response to therapy cannot be predicted on the basis of histology alone. Thus, nephrotic patients with primary FSGS should receive a trial of therapy irrespective of the histologic lesion when not contraindicated.Focal segmental glomerulosclerosis (FSGS) is a pattern of injury defined by a segmental scar, which involves some but not all glomeruli. When all of the secondary causes of this pattern of injury are eliminated, the remaining patients receive a diagnosis of primary FSGS. Although patients with primary FSGS may present with any level of proteinuria, clinical concern is greatest for those who present with nephrotic-range proteinuria because without treatment, they have an extremely poor prognosis, progressing to ESRD over the course of 3 to 6 yr (1,2). However, it is widely recognized that the prognosis in nephrotic patients with primary FSGS is significantly improved when remission of proteinuria is achieved. Because Ͼ50% of nephrotic adult patients with FSGS respond to an aggressive course of steroids, a trial of therapy has been recommended (1,3-5).Over the last 20 yr, in addition to the "c...

show abstract

The glomerular tip lesion: A previously undescribed type of segmental glomerular abnormality

Cited by 102 publications

References 11 publications

American Society of Nephrology Quiz and Questionnaire 2015: Glomerular Diseases

American Society of Nephrology Quiz and Questionnaire 2015: Glomerular Diseases

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

Focal Segmental Glomerulosclerosis in Nephrotic Adults

Contact Info

Product

Resources

About