Document Detail

Association of histologic variants in FSGS clinical trial with presenting features and outcomes.
MedLine Citation:
PMID:  23220425     Owner:  NLM     Status:  MEDLINE    
BACKGROUND AND OBJECTIVES: FSGS histologic variants have correlated with outcomes in retrospective studies. The FSGS Clinical Trial provided a unique opportunity to study the clinical impact of histologic variants in a well defined prospective cohort with steroid-resistant primary FSGS.
DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Renal biopsies of 138 FSGS Clinical Trial participants aged 2-38 years enrolled from 2004 to 2008 were analyzed using the Columbia classification by core pathologists. This study assessed the distribution of histologic variants and examined their clinical and biopsy characteristics and relationships to patient outcomes.
RESULTS: The distribution of histologic variants was 68% (n=94) FSGS not otherwise specified, 12% (n=16) collapsing, 10% (n=14) tip, 7% (n=10) perihilar, and 3% (n=4) cellular. Individuals with not otherwise specified FSGS were more likely to have subnephrotic proteinuria (P=0.01); 33% of teenagers and adults had tip or collapsing variants compared with 10% of children, and subjects with these variants had greater proteinuria and hypoalbuminemia than not otherwise specified patients. Tip variant had the strongest association with white race (86%) and the lowest pathologic injury scores, baseline creatinine, and rate of progression. Collapsing variant had the strongest association with black race (63%, P=0.03) and the highest pathologic injury scores (P=0.003), baseline serum creatinine (P=0.003), and rate of progression. At 3 years, 47% of collapsing, 20% of not otherwise specified, and 7% of tip variant patients reached ESRD (P=0.005).
CONCLUSIONS: This is the first prospective study with protocol-defined immunomodulating therapies confirming poor renal survival in collapsing variant and showing better renal survival in tip variant among steroid-resistant patients.
Vivette D D'Agati; Joan M Alster; J Charles Jennette; David B Thomas; James Pullman; Daniel A Savino; Arthur H Cohen; Debbie S Gipson; Jennifer J Gassman; Milena K Radeva; Marva M Moxey-Mims; Aaron L Friedman; Frederick J Kaskel; Howard Trachtman; Charles E Alpers; Agnes B Fogo; Tom H Greene; Cynthia C Nast
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Publication Detail:
Type:  Comparative Study; Journal Article; Multicenter Study; Randomized Controlled Trial; Research Support, N.I.H., Extramural     Date:  2012-12-06
Journal Detail:
Title:  Clinical journal of the American Society of Nephrology : CJASN     Volume:  8     ISSN:  1555-905X     ISO Abbreviation:  Clin J Am Soc Nephrol     Publication Date:  2013 Mar 
Date Detail:
Created Date:  2013-03-08     Completed Date:  2013-08-26     Revised Date:  2014-03-09    
Medline Journal Info:
Nlm Unique ID:  101271570     Medline TA:  Clin J Am Soc Nephrol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  399-406     Citation Subset:  IM    
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MeSH Terms
Adrenal Cortex Hormones / therapeutic use
African Americans
Age Factors
Biological Markers / blood
Child, Preschool
Creatinine / blood
Disease Progression
Drug Resistance
European Continental Ancestry Group
Glomerulosclerosis, Focal Segmental / drug therapy,  ethnology,  pathology*
Hypoalbuminemia / ethnology,  pathology
Immunosuppressive Agents / therapeutic use
Kaplan-Meier Estimate
Kidney / drug effects,  pathology*
Kidney Failure, Chronic / ethnology,  pathology
Predictive Value of Tests
Proportional Hazards Models
Prospective Studies
Proteinuria / ethnology,  pathology
Remission Induction
Risk Assessment
Risk Factors
Severity of Illness Index
Time Factors
Treatment Outcome
United States / epidemiology
Young Adult
Grant Support
Reg. No./Substance:
0/Adrenal Cortex Hormones; 0/Biological Markers; 0/Immunosuppressive Agents; AYI8EX34EU/Creatinine
Comment In:
Clin J Am Soc Nephrol. 2013 Mar;8(3):344-6   [PMID:  23430205 ]
Nat Rev Nephrol. 2013 Feb;9(2):65   [PMID:  23296292 ]

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