Clinical features and outcomes of IgA nephropathy with nephrotic syndrome.
OBJECTIVE
Nephrotic syndrome (NS) is a rare manifestation of IgA nephropathy (IgAN). Clinical characteristics and long-term outcomes of this condition have not yet been explored.


METHODS
A multicenter observational study was conducted between January 2000 and September 2010 in 1076 patients with biopsy-proven IgAN from four medical centers in Korea. The primary outcome was a doubling of the baseline serum creatinine concentration.


RESULTS
Of the 1076 patients, 100 (10.2%) presented with NS; 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 ≤1.2 mg/dl and a >50% decrease in proteinuria within 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.
Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
Jwa-Kyung
JK
Kim
Jeong Ho
JH
Kim
Sang Choel
SC
Lee
Ea Wha
EW
Kang
Tae Ik
TI
Chang
Sung Jin
SJ
Moon
Soo Young
SY
Yoon
Tae-Hyun
TH
Yoo
Shin-Wook
SW
Kang
Kyu Hun
KH
Choi
Dae Suk
DS
Han
Jeong Hae
JH
Kie
Beom Jin
BJ
Lim
Hyeon Joo
HJ
Jeong
Seung Hyeok
SH
Han
D000328:Adult; D015415:Biomarkers / Q000097:blood; D001706:Biopsy; D016009:Chi-Square Distribution; D003404:Creatinine / Q000097:blood; D005260:Female; D005922:Glomerulonephritis, IGA / Q000097:blood / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D053208:Kaplan-Meier Estimate; D007676:Kidney Failure, Chronic / Q000209:etiology; D008297:Male; D008875:Middle Aged; D009404:Nephrotic Syndrome / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000401:mortality; D011237:Predictive Value of Tests; D016016:Proportional Hazards Models; D012008:Recurrence; D012074:Remission Induction; D012075:Remission, Spontaneous; D056910:Republic of Korea; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D015854:Up-Regulation; D055815:Young Adult
D015415:Biomarkers; D007166:Immunosuppressive Agents; D003404:Creatinine