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Clinical Features and Histologic Predictors of Renal Outcome in Pauci-Immune Necrotizing Glomerulonephritis

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Background: Pauci-immune necrotizing glomerulonephritis (PING) is a relatively rare disease. Some patients may suffer from end-stage renal disease (ESRD) if there is a delayed diagnosis or it is left untreated. Kidney biopsy remains an important tool for early diagnosis and evaluation the extent of renal involvement. The purpose of this study is to analyze clinical and pathological prognostic markers in PING. Materials and Methods: Between July 1996 and July 2006, patients with biopsy-proven PING were enrolled. Clinical data including age, gender, body mass index (BMI), glomerular filtration rate (GFR), proteinuria, hematological data, immune profiles, clinical manifestations, and pathological findings were retrieved from medical charts. Result: Among 1570 biopsies, 27 cases (1.7%) were diagnosed as PING. Of the 27 patients, 20 (74%) were p-ANCA positive and 3(11%) were c-ANCA positive and 4(15%) were ANCA negative. The mean age was 54.3±15.2 years (range: 16 to 72 years). Malaise (77.8%) and pulmonary complaints (52%) were the most common extra-renal manifestations. During a mean follow up of 47±6 months after renal biopsy, 18 patients progressed to ESRD, while 9 patients remained dialysis-free. Two patients underwent successful renal transplantation 1-2 years after ESRD. When compared with each parameter separately, dialysis-independent patients had higher glomerular filtration rate (27.3±23.5 vs 11.3±15.0 ml/min, p=0.035), higher hemoglobin level (10.3±2.7 vs 8.2±2.4 g/dl, p=0.043), more percentage of normal glomeruli (34.6±20.5 vs 14.6±19.2%, p=0.009), and less interstitial fibrosis (13.9±8.6 vs 36.3±21.5%, p=0.001). The 1 and 5 year renal survival rates was 37.2% and 23.3%, respectively, while the patient survival rate were 75.8% and 54%, respectively. Conclusion: PING is a severe glomerulonephritis with a poor prognosis. Our study highlights the importance of kidney biopsy in diagnosis and predicting renal outcome of PING.

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