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

Objective To investigate the clinical and pathological characteristics of pauci-immune crescent glomerulonephritis (PICGN) in Chinese patients. Methods During 13 years (1985 - 1998), 6400 patients underwent non-transplanting renal biopsy. Twenty-four patients were diagnosed as PICGN. All clinical and laboratory data of these patients were collected from the patients' records and used for detailed analysis. The diagnosis is based on clinicopathologic findings. Results Of the 24 patients, 16 were females and 8 were males, with median age of 33 years (ranged 10 -76 years). Microscopic polyarteritis (MPA) (33.3%) and systemic vasculitis (8.3%) were the secondary diseases. The incidence of PICGN was 0.38% in renal biopsies and 22.9% in crescentic glomerulonephritis. Clinically, most patients (75.0%) showed rapidly progressive nephritis with enlarged kidneys. At onset, gross hematuria was noted in 58.3% of patients, hypertension in 45.8%, nephrotic syndrome in 41.7%, and oliguria in 25.0%. However, systemic symptoms were rare except for anemia. Pathologically, necrosis of glomerular capillaries (62.5%), infiltration of monocytes and neutrophil cells in glomeruli (66.7%), and vasculitis in the interstitium (53.3%) were observed. In addition, glomerulosclerosis was noted in 45.8%, severe tubular atrophy in 83.3% and interstitial fibrosis in 75.0%. Anti-neutrophil cytoplasmic antibodies (ANCAs) were positive in 52.2%. All patients except two received intensively immunosuppressive therapy. Sixteen patients were available for long-term follow up (median 29.8 months, range 8 - 92 months). Twelve of them had life-sustaining renal function, four had normal serum creatinine ( < 124 <mu>mol/L) and only 4 patients were dialysis-dependent. Conclusion PICGN is not rare in China. Early diagnosis and administration of immunosuppressive therapy, particularly in patients with rapidly progressive glomerulonephritis (RPGN), are important for good prognosis.