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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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Flow diagram of the study.
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Figure 1: Flow diagram of the study.

Mentions: A review of the medical records identified 147 patients who were diagnosed with primary FSGS between January 2004 and February 2013 by renal biopsy in Yonsei University Severance hospital and National Health Insurance Service Ilsan hospital in Korea. These patients did not have another glomerular disease or other conditions that were secondarily related to FSGS such as reflux, human immunodeficiency virus (HIV) infection, sickle cell anemia, surgical renal ablation, or solitary kidney. In addition, we confirmed that the patients were not exposed to heroin, lithium, calcineurin-inhibitor, or pamidronate prior to diagnosis. There was no familial FSGS in our medical records. Among these patients, 36 patients were excluded for following reasons; age < 18 years (n = 3), follow-up duration < 6 months (n = 21), inadequate number of glomeruli < 7 (n = 6), and 24-h proteinuria < 0.5 g/day (n = 6). Finally, 111 FSGS patients were included in the analysis (Figure 1).


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)

Flow diagram of the study.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 1: Flow diagram of the study.
Mentions: A review of the medical records identified 147 patients who were diagnosed with primary FSGS between January 2004 and February 2013 by renal biopsy in Yonsei University Severance hospital and National Health Insurance Service Ilsan hospital in Korea. These patients did not have another glomerular disease or other conditions that were secondarily related to FSGS such as reflux, human immunodeficiency virus (HIV) infection, sickle cell anemia, surgical renal ablation, or solitary kidney. In addition, we confirmed that the patients were not exposed to heroin, lithium, calcineurin-inhibitor, or pamidronate prior to diagnosis. There was no familial FSGS in our medical records. Among these patients, 36 patients were excluded for following reasons; age < 18 years (n = 3), follow-up duration < 6 months (n = 21), inadequate number of glomeruli < 7 (n = 6), and 24-h proteinuria < 0.5 g/day (n = 6). Finally, 111 FSGS patients were included in the analysis (Figure 1).

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