Open Access Research article

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

Young Eun Kwon1, Seung Hyeok Han1, Jeong Hae Kie2, Seong Yeong An1, Yung Ly Kim1, Kyoung Sook Park1, Ki Heon Nam1, Ah Young Leem1, Hyung Jung Oh1, Jung Tak Park1, Tae Ik Chang3, Ea Wha Kang3, Shin-Wook Kang1, Kyu Hun Choi1, Beom Jin Lim4, Hyeon Joo Jeong4 and Tae-Hyun Yoo1*

Author Affiliations

1 Department of Internal Medicine, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-Gu, Seoul 120-752, Korea

2 Department of Pathology, NHIS Medical Center Ilsan Hospital, Goyang-shi, Gyeonggi-do, Korea

3 Department of Internal Medicine, NHIS Medical Center Ilsan Hospital, Goyang-shi, Gyeonggi-do, Korea

4 Department of Pathology, Yonsei University College of Medicine, Seoul, Korea

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BMC Nephrology 2014, 15:52  doi:10.1186/1471-2369-15-52

Published: 25 March 2014

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 (Pā€‰=ā€‰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.

Keywords:
Focal segmental glomerulosclerosis; Pathology; Outcome