Focal and segmental glomerulosclerosis: Clinical and kidney biopsy correlations

Sanjeev Sethi, Ladan Zand, Samih H. Nasr, Richard J. Glassock, Fernando C. Fervenza

Research output: Contribution to journalArticle

24 Scopus citations

Abstract

Background. Primary focal segmental glomerulosclerosis (FSGS) is a common glomerular disease in adults and ranks among the top causes of a primary glomerular disease causing end-stage renal disease (ESRD). Primary FSGS is, however, a diagnosis of exclusion and distinction between primary versus secondary FSGS is not always obvious, resulting in a number of patients with secondary FSGS undergoing unnecessary immunosuppressive therapy. Methods. We reviewed the Mayo Clinic Renal Pathology Database for patients with a diagnosis of FSGS on native renal biopsy and divided the patients into nephrotic syndrome-associated (NSassociated) and non-nephrotic syndrome-associated (NNS-associated) FSGS as a first approximation followed by dividing the lesion according to the degree of foot process effacement (FPE) on electron microscopy (EM) examination. Results. A total of 41 patients with FSGS with complete evaluation were identified. Of these, 18 were classified as having NS and 23 were classified as having NNS. Baseline characteristics (age, gender, body mass index, serum creatinine and hematuria) were not different between the groups. All of the patients with NS showed diffuse FPE ranging from 80 to 100% (mean 96%). On the other hand, of the 23 patients in the NNS group, 22 had segmental FPE and showed patchy effacement, with all cases showing 20-60% FPE (mean of 48%). Conclusion. Adult patients presenting with NS, an FSGS lesion on LM, extensive FPE (≥80%) on EM examination and no risk factors associated with secondary FSGS are likely to have primary FSGS. Conversely, the absence of NS in a patient with segmental FPE on EM strongly suggests a secondary FSGS. Dividing FSGS into the presence or absence of NS together with the degree of FPE on EM examination is more helpful as it provides a more practical way to separate patients into cases of primary versus secondary FSGS.

Original languageEnglish (US)
Pages (from-to)531-537
Number of pages7
JournalClinical Kidney Journal
Volume7
Issue number6
DOIs
StatePublished - Dec 1 2014
Externally publishedYes

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Keywords

  • Focal and segmental glomerulosclerosis
  • Nephrotic range
  • Nephrotic syndrome
  • Primary
  • Secondary

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