Clinical features and outcomes of IgA nephropathy with nephrotic syndrome

Jwa Kyung Kim, Jeong Ho Kim, Sang Choel Lee, Ea Wha Kang, Tae Ik Chang, Sung Jin Moon, Soo Young Yoon, TaeHyun Yoo, Shin-Wook Kang, Kyu Hun Choi, Dae Suk Han, Jeong Hae Kie, Beom Jin Lim, Hyeon Joo Jeong, SeungHyeok Han

Research output: Contribution to journalArticle

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Abstract

Background and objectives: Nephrotic syndrome (NS) is a raremanifestation of IgA nephropathy (IgAN). Clinical characteristics and long-term outcomes of this condition have not yet been explored. Design, setting, participants, & measurements: A multicenter observational study was conducted between January 2000 and September 2010 in 1076 patients with biopsy-proven IgANfromfourmedical centers in Korea. The primary outcome was a doubling of the baseline serum creatinine concentration. ResultsOf the 1076 patients, 100 (10.2%) presentedwithNS; complete remission (CR), partial remission (PR), and no response (NR) occurred in 48 (48%), 32 (32%), and 20 (20%) patients, respectively. During the median follow-up of 45.2 months, 24 patients (24%) in the NS group reached the primary endpoint compared with 63 (7.1%) in the non-NS group (P < 0.001). The risk of reaching the primary endpoint was significantly higher in the PR (P=0.04) and NR groups (P < 0.001) than in the CR group. Among patients with NS, 24 (24%) underwent spontaneous remission (SR). SR occurred more frequently in female patients and in patients with serum creatinine levels ≤1.2mg/dl and a >50%decrease in proteinuriawithin 3 months after NS onset.None of the patients with SR reached the primary endpoint and they had fewer relapses during follow-up. Conclusions: This study demonstrated that the prognosis of NS in IgAN was not favorable unless PR or CR was achieved. In addition, SR was more common than expected, particularly in patients with preserved kidney function and spontaneous decrease in proteinuria shortly after NS onset.

Original languageEnglish
Pages (from-to)427-436
Number of pages10
JournalClinical Journal of the American Society of Nephrology
Volume7
Issue number3
DOIs
Publication statusPublished - 2012 Mar 1

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Nephrotic Syndrome
Immunoglobulin A
Korea
Proteinuria
Multicenter Studies
Observational Studies
Creatinine
Kidney
Biopsy
Recurrence
Serum

All Science Journal Classification (ASJC) codes

  • Epidemiology
  • Critical Care and Intensive Care Medicine
  • Nephrology
  • Transplantation

Cite this

Kim, Jwa Kyung ; Kim, Jeong Ho ; Lee, Sang Choel ; Kang, Ea Wha ; Chang, Tae Ik ; Moon, Sung Jin ; Yoon, Soo Young ; Yoo, TaeHyun ; Kang, Shin-Wook ; Choi, Kyu Hun ; Han, Dae Suk ; Kie, Jeong Hae ; Lim, Beom Jin ; Jeong, Hyeon Joo ; Han, SeungHyeok. / Clinical features and outcomes of IgA nephropathy with nephrotic syndrome. In: Clinical Journal of the American Society of Nephrology. 2012 ; Vol. 7, No. 3. pp. 427-436.
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title = "Clinical features and outcomes of IgA nephropathy with nephrotic syndrome",
abstract = "Background and objectives: Nephrotic syndrome (NS) is a raremanifestation of IgA nephropathy (IgAN). Clinical characteristics and long-term outcomes of this condition have not yet been explored. Design, setting, participants, & measurements: A multicenter observational study was conducted between January 2000 and September 2010 in 1076 patients with biopsy-proven IgANfromfourmedical centers in Korea. The primary outcome was a doubling of the baseline serum creatinine concentration. ResultsOf the 1076 patients, 100 (10.2{\%}) presentedwithNS; complete remission (CR), partial remission (PR), and no response (NR) occurred in 48 (48{\%}), 32 (32{\%}), and 20 (20{\%}) patients, respectively. During the median follow-up of 45.2 months, 24 patients (24{\%}) in the NS group reached the primary endpoint compared with 63 (7.1{\%}) in the non-NS group (P < 0.001). The risk of reaching the primary endpoint was significantly higher in the PR (P=0.04) and NR groups (P < 0.001) than in the CR group. Among patients with NS, 24 (24{\%}) underwent spontaneous remission (SR). SR occurred more frequently in female patients and in patients with serum creatinine levels ≤1.2mg/dl and a >50{\%}decrease in proteinuriawithin 3 months after NS onset.None of the patients with SR reached the primary endpoint and they had fewer relapses during follow-up. Conclusions: This study demonstrated that the prognosis of NS in IgAN was not favorable unless PR or CR was achieved. In addition, SR was more common than expected, particularly in patients with preserved kidney function and spontaneous decrease in proteinuria shortly after NS onset.",
author = "Kim, {Jwa Kyung} and Kim, {Jeong Ho} and Lee, {Sang Choel} and Kang, {Ea Wha} and Chang, {Tae Ik} and Moon, {Sung Jin} and Yoon, {Soo Young} and TaeHyun Yoo and Shin-Wook Kang and Choi, {Kyu Hun} and Han, {Dae Suk} and Kie, {Jeong Hae} and Lim, {Beom Jin} and Jeong, {Hyeon Joo} and SeungHyeok Han",
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Kim, JK, Kim, JH, Lee, SC, Kang, EW, Chang, TI, Moon, SJ, Yoon, SY, Yoo, T, Kang, S-W, Choi, KH, Han, DS, Kie, JH, Lim, BJ, Jeong, HJ & Han, S 2012, 'Clinical features and outcomes of IgA nephropathy with nephrotic syndrome', Clinical Journal of the American Society of Nephrology, vol. 7, no. 3, pp. 427-436. https://doi.org/10.2215/CJN.04820511

Clinical features and outcomes of IgA nephropathy with nephrotic syndrome. / Kim, Jwa Kyung; Kim, Jeong Ho; Lee, Sang Choel; Kang, Ea Wha; Chang, Tae Ik; Moon, Sung Jin; Yoon, Soo Young; Yoo, TaeHyun; Kang, Shin-Wook; Choi, Kyu Hun; Han, Dae Suk; Kie, Jeong Hae; Lim, Beom Jin; Jeong, Hyeon Joo; Han, SeungHyeok.

In: Clinical Journal of the American Society of Nephrology, Vol. 7, No. 3, 01.03.2012, p. 427-436.

Research output: Contribution to journalArticle

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T1 - Clinical features and outcomes of IgA nephropathy with nephrotic syndrome

AU - Kim, Jwa Kyung

AU - Kim, Jeong Ho

AU - Lee, Sang Choel

AU - Kang, Ea Wha

AU - Chang, Tae Ik

AU - Moon, Sung Jin

AU - Yoon, Soo Young

AU - Yoo, TaeHyun

AU - Kang, Shin-Wook

AU - Choi, Kyu Hun

AU - Han, Dae Suk

AU - Kie, Jeong Hae

AU - Lim, Beom Jin

AU - Jeong, Hyeon Joo

AU - Han, SeungHyeok

PY - 2012/3/1

Y1 - 2012/3/1

N2 - Background and objectives: Nephrotic syndrome (NS) is a raremanifestation of IgA nephropathy (IgAN). Clinical characteristics and long-term outcomes of this condition have not yet been explored. Design, setting, participants, & measurements: A multicenter observational study was conducted between January 2000 and September 2010 in 1076 patients with biopsy-proven IgANfromfourmedical centers in Korea. The primary outcome was a doubling of the baseline serum creatinine concentration. ResultsOf the 1076 patients, 100 (10.2%) presentedwithNS; complete remission (CR), partial remission (PR), and no response (NR) occurred in 48 (48%), 32 (32%), and 20 (20%) patients, respectively. During the median follow-up of 45.2 months, 24 patients (24%) in the NS group reached the primary endpoint compared with 63 (7.1%) in the non-NS group (P < 0.001). The risk of reaching the primary endpoint was significantly higher in the PR (P=0.04) and NR groups (P < 0.001) than in the CR group. Among patients with NS, 24 (24%) underwent spontaneous remission (SR). SR occurred more frequently in female patients and in patients with serum creatinine levels ≤1.2mg/dl and a >50%decrease in proteinuriawithin 3 months after NS onset.None of the patients with SR reached the primary endpoint and they had fewer relapses during follow-up. Conclusions: This study demonstrated that the prognosis of NS in IgAN was not favorable unless PR or CR was achieved. In addition, SR was more common than expected, particularly in patients with preserved kidney function and spontaneous decrease in proteinuria shortly after NS onset.

AB - Background and objectives: Nephrotic syndrome (NS) is a raremanifestation of IgA nephropathy (IgAN). Clinical characteristics and long-term outcomes of this condition have not yet been explored. Design, setting, participants, & measurements: A multicenter observational study was conducted between January 2000 and September 2010 in 1076 patients with biopsy-proven IgANfromfourmedical centers in Korea. The primary outcome was a doubling of the baseline serum creatinine concentration. ResultsOf the 1076 patients, 100 (10.2%) presentedwithNS; complete remission (CR), partial remission (PR), and no response (NR) occurred in 48 (48%), 32 (32%), and 20 (20%) patients, respectively. During the median follow-up of 45.2 months, 24 patients (24%) in the NS group reached the primary endpoint compared with 63 (7.1%) in the non-NS group (P < 0.001). The risk of reaching the primary endpoint was significantly higher in the PR (P=0.04) and NR groups (P < 0.001) than in the CR group. Among patients with NS, 24 (24%) underwent spontaneous remission (SR). SR occurred more frequently in female patients and in patients with serum creatinine levels ≤1.2mg/dl and a >50%decrease in proteinuriawithin 3 months after NS onset.None of the patients with SR reached the primary endpoint and they had fewer relapses during follow-up. Conclusions: This study demonstrated that the prognosis of NS in IgAN was not favorable unless PR or CR was achieved. In addition, SR was more common than expected, particularly in patients with preserved kidney function and spontaneous decrease in proteinuria shortly after NS onset.

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