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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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Related in: MedlinePlus

Kaplan-Meier plots for (A) complete remission (CR) and (B) complete or partial remission (PR). Patients with tip lesion had higher probability of achieving CR and CR or PR.
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Figure 3: Kaplan-Meier plots for (A) complete remission (CR) and (B) complete or partial remission (PR). Patients with tip lesion had higher probability of achieving CR and CR or PR.

Mentions: In terms of remission rates, CR and PR were achieved in 29 (26.1%) and 35 (31.5%) patients, respectively. CR or PR was attained in 16 (80.0%) patients with tip variant compared to 38 (54.3%) with NOS variant (P = 0.038) and 7 (41.2%) with perihilar variant (P = 0.015) (Table 2). In addition, CR was more frequently achieved in patients with tip variant than those with NOS (P = 0.018) or perihilar (P = 0.040) variants. A higher rate of PR was also observed in patients with tip variant compared to those with other variants, but did not reach statistical significance. Kaplan-Meier plots also produced the same results (Figure 3A and B). In unadjusted Cox models, tip variant was associated with a significantly increased probability of achieving CR (HR, 2.616; 95% CI, 1.184-5.778; P = 0.017) and CR or PR (HR, 1.809; 95% CI, 1.007-3.250; P = 0.047). Multivariable analysis adjusted for age, sex, eGFR, proteinuria, and immunosuppression showed that tip variant conferred a 2.4-fold higher probability of achieving CR (P = 0.044, Table 4). A similar association was also observed when CR or PR was entered together as a dependent outcome variable, but the result was not statistically significant.


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) complete remission (CR) and (B) complete or partial remission (PR). Patients with tip lesion had higher probability of achieving CR and CR or PR.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 3: Kaplan-Meier plots for (A) complete remission (CR) and (B) complete or partial remission (PR). Patients with tip lesion had higher probability of achieving CR and CR or PR.
Mentions: In terms of remission rates, CR and PR were achieved in 29 (26.1%) and 35 (31.5%) patients, respectively. CR or PR was attained in 16 (80.0%) patients with tip variant compared to 38 (54.3%) with NOS variant (P = 0.038) and 7 (41.2%) with perihilar variant (P = 0.015) (Table 2). In addition, CR was more frequently achieved in patients with tip variant than those with NOS (P = 0.018) or perihilar (P = 0.040) variants. A higher rate of PR was also observed in patients with tip variant compared to those with other variants, but did not reach statistical significance. Kaplan-Meier plots also produced the same results (Figure 3A and B). In unadjusted Cox models, tip variant was associated with a significantly increased probability of achieving CR (HR, 2.616; 95% CI, 1.184-5.778; P = 0.017) and CR or PR (HR, 1.809; 95% CI, 1.007-3.250; P = 0.047). Multivariable analysis adjusted for age, sex, eGFR, proteinuria, and immunosuppression showed that tip variant conferred a 2.4-fold higher probability of achieving CR (P = 0.044, Table 4). A similar association was also observed when CR or PR was entered together as a dependent outcome variable, but the result was not statistically significant.

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