[Clinical and pathological characteristics in Chinese patients with pauci-immune crescentic glomerulonephritis]

We investigated pauci-immune crescentic glomerulonephritis (PICGN) in Chinese patients, During 11 years (1985-1996), 5,800 patients underwent renal biopsy in Nanjing Jinling Hospital. Eighteen patients were diagnosed as having PICGN. They were 11 women and 7 men with median age of 29 (range 10-71 years). Polyarteritis (33.3%) and polyarteritis nodosa (11.1%) were the secondary diseases. The incidence of PICGN was 0.31% in renal biopsies and 23.10% in crescentic glomerulonephritis. Clinically, most patients (83.3%) showed rapidly progressive nephritis with enlarged kidneys. Gross hemoturia was noted in 61.1% patients, hypertension 44.4%, nephrotic syndrome 44.4%, and oligria 33.3%. However, systemic symptoms were rare except anemia. Pathologically, we observed necrosis of glomerular capillaries (61.1%), infiltration of monocytes and neutrophil cells in glomeruli (44.4%), and vasculitis in interstitium (55.5%), in addition to glomerulosclerosis (44.4%), severe tubular atrophy (83.3%) and interstitial fibrosis (72.2%). Antineutrophil cytoplasmic antibodies (ANCA) was positive in 41.7%. All patients except one received intensively immunosuppressive therapy. 12 patients were subjected to long-term follow up (median 25.9, range 8-72 months). Only three patients were dialysis-dependent, while none died.