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

Kwon YE, Han SH, Kie JH, An SY, Kim YL, Park KS, Nam KH, Leem AY, Oh HJ, Park JT, Chang TI, Kang EW, Kang SW, Choi KH, Lim BJ, Jeong HJ, Yoo TH - BMC Nephrol (2014)

Bottom Line: 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).Although pathologic variants were not associated with overall outcome, the tip variant exhibited favorable outcome in terms of achieving remission.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Internal Medicine, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-Gu, Seoul 120-752, Korea. yoosy0316@yuhs.ac.

ABSTRACT

Background: Many 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.

Methods: This 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).

Results: There 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).

Conclusion: Similar 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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Kaplan-Meier plots for (A) overall event free renal survival and (B) event free renal survival by FSGS pathologic variants. Overall 5-renal survival rate was 76.8%. There were no significant difference in renal survival rates between patients with NOS, perihilar, and tip variants.
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Figure 2: Kaplan-Meier plots for (A) overall event free renal survival and (B) event free renal survival by FSGS pathologic variants. Overall 5-renal survival rate was 76.8%. There were no significant difference in renal survival rates between patients with NOS, perihilar, and tip variants.

Mentions: Median follow up duration was 34.5 months and twenty-seven patients were followed up more than 5 years. Sixteen (14.4%) patients reached the composite of D-sCr or ESRD and the overall 5-year event-free renal survival rate was 76.8% (Figure 2A). Twelve (17.1%) patients with NOS variant reached the composite primary outcome compared to 2 (11.8%) with perihilar variant and 1 (5.0%) with tip variant (Table 2, P = 0.370). A Kaplan-Meier curve also showed that overall renal survival rates were not statistically significant between patients with NOS, tip, and perihilar variants (Figure 2B, P = 0.616). Multivariable analysis after adjustment of age, sex, hypertension, eGFR, proteinuria, and immunosuppression showed no difference in the development of primary outcome among the three groups (Table 3). Among three patients with cellular variant, two patients showed kidney impairment at presentation (eGFR of 24.6 and 36.2 mL/min/1.73 m2, respectively) and one reached the composite outcome. At two patients with cellular variant showed UPCR 16.04 and 3.94 g/g at the time of biopsy, UPCR were reduced to 2.84 and 1.93 g/g after treatment of corticosteroid or cyclosporine, respectively. One patient with collapsing variant presented nephrotic syndrome and exhibited an eGFR of 38 mL/min/1.73 m2 and UPCR of 16.7 g/g at the time of biopsy. Twelve-month corticosteroid treatment resulted in reduction of UPCR to 1.43 g/g and improvement of eGFR to 38 mL/min/1.73 m2 in this patient.


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

Kwon YE, Han SH, Kie JH, An SY, Kim YL, Park KS, Nam KH, Leem AY, Oh HJ, Park JT, Chang TI, Kang EW, Kang SW, Choi KH, Lim BJ, Jeong HJ, Yoo TH - BMC Nephrol (2014)

Kaplan-Meier plots for (A) overall event free renal survival and (B) event free renal survival by FSGS pathologic variants. Overall 5-renal survival rate was 76.8%. There were no significant difference in renal survival rates between patients with NOS, perihilar, and tip variants.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4230406&req=5

Figure 2: Kaplan-Meier plots for (A) overall event free renal survival and (B) event free renal survival by FSGS pathologic variants. Overall 5-renal survival rate was 76.8%. There were no significant difference in renal survival rates between patients with NOS, perihilar, and tip variants.
Mentions: Median follow up duration was 34.5 months and twenty-seven patients were followed up more than 5 years. Sixteen (14.4%) patients reached the composite of D-sCr or ESRD and the overall 5-year event-free renal survival rate was 76.8% (Figure 2A). Twelve (17.1%) patients with NOS variant reached the composite primary outcome compared to 2 (11.8%) with perihilar variant and 1 (5.0%) with tip variant (Table 2, P = 0.370). A Kaplan-Meier curve also showed that overall renal survival rates were not statistically significant between patients with NOS, tip, and perihilar variants (Figure 2B, P = 0.616). Multivariable analysis after adjustment of age, sex, hypertension, eGFR, proteinuria, and immunosuppression showed no difference in the development of primary outcome among the three groups (Table 3). Among three patients with cellular variant, two patients showed kidney impairment at presentation (eGFR of 24.6 and 36.2 mL/min/1.73 m2, respectively) and one reached the composite outcome. At two patients with cellular variant showed UPCR 16.04 and 3.94 g/g at the time of biopsy, UPCR were reduced to 2.84 and 1.93 g/g after treatment of corticosteroid or cyclosporine, respectively. One patient with collapsing variant presented nephrotic syndrome and exhibited an eGFR of 38 mL/min/1.73 m2 and UPCR of 16.7 g/g at the time of biopsy. Twelve-month corticosteroid treatment resulted in reduction of UPCR to 1.43 g/g and improvement of eGFR to 38 mL/min/1.73 m2 in this patient.

Bottom Line: 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).Although pathologic variants were not associated with overall outcome, the tip variant exhibited favorable outcome in terms of achieving remission.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Internal Medicine, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-Gu, Seoul 120-752, Korea. yoosy0316@yuhs.ac.

ABSTRACT

Background: Many 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.

Methods: This 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).

Results: There 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).

Conclusion: Similar 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.

Show MeSH
Related in: MedlinePlus