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PLoS One. 2019 Apr 19;14(4):e0215217. doi: 10.1371/journal.pone.0215217. eCollection 2019.

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

Kim K1, Son HE1, Ryu JY1, Lee H2,3,4, Han SH2,5, Ryu DR2,6, Paik JH7, Kim S1, Na KY1,8, Chae DW1,8, Chin HJ1,2,4,8, Oh SW2,9.

Author information

1
Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Republic of Korea.
2
Korean GlomeuloNephritis Study Group, Korean Society of Nephrology, Seoul, Republic of Korea.
3
Department of Internal Medicine, Seoul National University Hospital, Seoul, Republic of Korea.
4
Kidney Research Institute, Seoul National University College of Medicine, Seoul, Republic of Korea.
5
Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of Korea.
6
Department of Internal Medicine, School of Medicine, Ewha Woman's University, Seoul, Republic of Korea.
7
Department of Pathology, Seoul National University Bundang Hospital, Seongnam, Republic of Korea.
8
Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea.
9
Department of Internal Medicine, Korea University College of Medicine and Korea University Anam Hospital, Seoul, Republic of Korea.

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 end-stage 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.

Conflict of interest statement

The authors have declared that no competing interests exist.

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