Clinical and pathological features of immunoglobulin A nephropathy patients with nephrotic syndrome.

IgA nephropathy (IgAN) is the most common glomerulonephritis worldwide. The classic manifestation of IgAN is episodic hematuria and proteinuria. Nephrotic syndrome (NS) is not very common in IgAN, reported to occur in only 5-10% of IgAN patients. However, the clinical and pathological characteristics and long-term outcomes of patients with NS-IgAN at onset remain unknown. A retrospective study was conducted, enrolling 1165 patients with biopsy-proven IgAN from West China Hospital in 2008-2015. Patients with renal biopsy of minimal change disease with mesangial IgA deposits were excluded. The renal endpoint was defined as 50% decrease in eGFR or progressing into end-stage renal disease (ESRD). A total of 1165 patients were enrolled with average age of 34.58, and 171 (14.7%) patients were presented with NS. Comparing NS and non-NS groups, significance differences were shown in hypertension (HTN), 24-h urine protein, serum albumin, serum creatine, eGFR and uric acid. NS group had severe pathological changes such as endocapillary hypercellularity, tubular atrophy or interstitial fibrosis and crescent, but less segmental glomerulosclerosis or adhesion and global sclerosis. During the average follow-up of 44.27 months, 29.8% (51/171) NS patients and 15.8% (157/994) non-NS patients progressed to the renal endpoint. 5-year renal survival rates were 73.1% and 87.8% (P < 0.001) in NS and non-NS groups, respectively. This study demonstrated that IgAN patients with NS had higher serum creatine, lower eGFR, lower uric acid, more acute lesions and poor prognosis. NS was an independent risk factor for progression to the renal endpoint.