C1q nephropathy in adults is a form of focal segmental glomerulosclerosis in terms of clinical characteristics

Kipyo Kim, Hyung Eun Son, Ji Young Ryu, Hajeong Lee, Seung Hyeok Han, Dong Ryeol Ryu, Jin Ho Paik, Sejoong Kim, Ki Young Na, Dong Wan Chae, Ho Jun Chin, Se Won Oh

Research output: Contribution to journalArticle

Abstract

Although C1q nephropathy (C1qN) was introduced three decades ago, the clinical significance and renal outcomes of C1qN remain unclear. This study aimed to evaluate the clinical characteristics of C1qN, including renal outcomes, by performing a matched comparison within a multicenter cohort. We enrolled 6,413 adult patients who underwent kidney biopsy between January 2000 and January 2018 at three tertiary hospitals in Korea. We compared the clinical characteristics of 23 patients with C1qN with those of patients with focal segmental glomerulosclerosis (FSGS) or minimal change disease (MCD) who were matched by age, sex, diabetic status, and a period of biopsy. Histological and clinical parameters in patients with C1qN were also evaluated according to the different pathological phenotypes. For a mean follow-up period of 92 months, 4 patients with C1qN (17.4%) developed endstage renal disease (ESRD). None of the matched patients with MCD had ESRD, but 7 (30.4%) of patients with FSGS progressed to ESRD, which was not different from that of C1qN patients (p = 0.491). Laboratory and pathological findings, except segmental glomerulosclerosis, were not notably different between FSGS and C1qN. The presence of segmental glomerulosclerosis, mesangial hypercellularity, and podocyte effacement did not affect both the short- and long-term renal outcomes in patients with C1qN. Our study showed that the renal outcomes of C1qN are comparable with those of FSGS, and not with MCD. Specific pathological findings, including segmental glomerulosclerosis in C1qN, were not associated with renal outcomes, which may suggest homogeneity in the clinical features of C1qN.

Original languageEnglish
Article numbere0215217
JournalPLoS ONE
Volume14
Issue number4
DOIs
Publication statusPublished - 2019 Apr 1

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Focal Segmental Glomerulosclerosis
kidney diseases
Kidney
Lipoid Nephrosis
Biopsy
kidneys
Podocytes
Korea
biopsy
Tertiary Care Centers
Phenotype

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)
  • Agricultural and Biological Sciences(all)

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C1q nephropathy in adults is a form of focal segmental glomerulosclerosis in terms of clinical characteristics. / Kim, Kipyo; Son, Hyung Eun; Ryu, Ji Young; Lee, Hajeong; Han, Seung Hyeok; Ryu, Dong Ryeol; Paik, Jin Ho; Kim, Sejoong; Na, Ki Young; Chae, Dong Wan; Chin, Ho Jun; Oh, Se Won.

In: PLoS ONE, Vol. 14, No. 4, e0215217, 01.04.2019.

Research output: Contribution to journalArticle

Kim, K, Son, HE, Ryu, JY, Lee, H, Han, SH, Ryu, DR, Paik, JH, Kim, S, Na, KY, Chae, DW, Chin, HJ & Oh, SW 2019, 'C1q nephropathy in adults is a form of focal segmental glomerulosclerosis in terms of clinical characteristics', PLoS ONE, vol. 14, no. 4, e0215217. https://doi.org/10.1371/journal.pone.0215217
Kim, Kipyo ; Son, Hyung Eun ; Ryu, Ji Young ; Lee, Hajeong ; Han, Seung Hyeok ; Ryu, Dong Ryeol ; Paik, Jin Ho ; Kim, Sejoong ; Na, Ki Young ; Chae, Dong Wan ; Chin, Ho Jun ; Oh, Se Won. / C1q nephropathy in adults is a form of focal segmental glomerulosclerosis in terms of clinical characteristics. In: PLoS ONE. 2019 ; Vol. 14, No. 4.
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AU - Son, Hyung Eun

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AU - Han, Seung Hyeok

AU - Ryu, Dong Ryeol

AU - Paik, Jin Ho

AU - Kim, Sejoong

AU - Na, Ki Young

AU - Chae, Dong Wan

AU - Chin, Ho Jun

AU - Oh, Se Won

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AB - Although C1q nephropathy (C1qN) was introduced three decades ago, the clinical significance and renal outcomes of C1qN remain unclear. This study aimed to evaluate the clinical characteristics of C1qN, including renal outcomes, by performing a matched comparison within a multicenter cohort. We enrolled 6,413 adult patients who underwent kidney biopsy between January 2000 and January 2018 at three tertiary hospitals in Korea. We compared the clinical characteristics of 23 patients with C1qN with those of patients with focal segmental glomerulosclerosis (FSGS) or minimal change disease (MCD) who were matched by age, sex, diabetic status, and a period of biopsy. Histological and clinical parameters in patients with C1qN were also evaluated according to the different pathological phenotypes. For a mean follow-up period of 92 months, 4 patients with C1qN (17.4%) developed endstage renal disease (ESRD). None of the matched patients with MCD had ESRD, but 7 (30.4%) of patients with FSGS progressed to ESRD, which was not different from that of C1qN patients (p = 0.491). Laboratory and pathological findings, except segmental glomerulosclerosis, were not notably different between FSGS and C1qN. The presence of segmental glomerulosclerosis, mesangial hypercellularity, and podocyte effacement did not affect both the short- and long-term renal outcomes in patients with C1qN. Our study showed that the renal outcomes of C1qN are comparable with those of FSGS, and not with MCD. Specific pathological findings, including segmental glomerulosclerosis in C1qN, were not associated with renal outcomes, which may suggest homogeneity in the clinical features of C1qN.

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