The clinical and pathological characteristics of Chinese patients with pauci-immune crescentic glomerulonephritis

In the present study, we investigated pauci-immune crescentic glomerulonephritis (PICGN) in Chinese patients. During 13 years (1985-98), 6400 patients underwent non-transplanting renal biopsy in the Nanjing Jinling Hospital. Twenty-four patients were diagnosed as having PICGN. They were 16 women and eight men with a median age of 33 (range 10-76 years). Microscopic polyarteritis (33.3%) and polyarteritis nodosa (8.3%) were the secondary diseases. The incidence of PICGN was 0.37% in renal biopsies and 22.9% in crescentic glomerulonephritis. Clinically, most patients (75.0%) showed rapidly progressive nephritis with enlarged kidneys. Onset gross haematuria was noted in 58.3% of the patients, hypertension 45.8%, nephrotic syndrome 41.7%, and oliguria 25.0%. However, systemic symptoms were rare except anaemia. Pathologically, we observed necrosis of glomerular capillaries (62.5%), infiltration of monocytes and neutrophil cells in glomeruli (66.7%), and vasculitis in interstitium (53.3%), in addition to glomerulosclerosis more than 50% (45.8%), severe tubular atrophy (83.3%) and interstitial fibrosis (75.0%). Antineutrophil cytoplasmic antibodies were positive in 52.2%. All patients except two received intensively immunosuppressive therapy. Sixteen patients were subjected to long-term follow up (median 29.8, range 8-92 months), 12 of them had life-sustaining renal function, four had normal range of serum creatinine (< 124 mu mol/L), only four patients were dialysis dependent.