Clinical Features and Outcomes of IgA Nephropathy with Nephrotic Syndrome

Abstract

Summary Background and objectives 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. Design, setting, participants, & measurements 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.

IgA Nephropathy with Minimal Change Disease

“…Kim et al (33) concluded that IgAN patients with NS have a worse prognosis, but it is notable that many of the patients had significant proliferative lesions. In fact, only four patients had the exclusively mesangial proliferative disease and diffuse podocyte foot process effacement that was characteristic of patients in our cohort (33). In the series reported herein, all 17 patients showed a response to corticosteroid therapy.…”

Japan Renal Biopsy Registry and Japan Kidney Disease Registry: Committee Report for 2009 and 2010

“…Previously, the best single predictor for renal deterioration was severe proteinuria on urine dipstick testing (C100 mg/dL), followed by hypoalbuminemia, mild hematuria, serum total protein levels, diastolic blood pressure, and histological grade, in a cohort study with 10 years follow-up from 1995 in Japan, the cohort of which exhibited a younger median age (27.7 years) and a peak distribution of age ranges in the teens [11,12]. A recent report suggested that IgAN with nephrotic syndrome had a worse renal outcome compared to IgAN with non-nephrotic syndrome unless partial or complete remission was achieved [13]. Further studies are necessary to elucidate the risk factors or predictors for renal deterioration in IgAN in the present era utilizing the J-RBR, possibly as part of a new secondary clinical study.…”

Crescents and Global Glomerulosclerosis in Chinese IgA Nephropathy Patients: A Five-Year Follow-Up

“…Immunoglobulin A nephropathy (IgAN), the most common form of glomerulonephritis, is characterized by the accumulation of IgA immune complex deposits in the mesangium of the kidney [1]. The Oxford classification of IgAN has been widely adopted since 2009, which includes several definitive histological features: mesangial hypercellularity (M), endocapillary proliferation (E), segmental sclerosis or adhesion (S) and tubular atrophy/interstitial fibrosis (T) [2].…”