신질환 환자에서 Anti - Neutrophil Cytoplasmic Autoantibody 의 의의 = Anti - Neutrophil Cytoplasmic Autoantibody in Renal Disease

다국어 초록 (Multilingual Abstract)

Objectives: To clarify the spectrum of renal disease in patients with positive anti-neutrophil cytoplasmic autoantibody (ANCA); to compare the occurrence of crescent according to the type of ANCA; to assess the association between change of renal function and serial ANCA titers. Methods: Forty-five patients with biopsy proven renal disease underwent determination of ANCA by indirect immunofluorescent technique. Titers were repeated in 6 patients with median duration of 12 months (range, 3 to 28 months). Results: ANCA was detected in 17 of 45 patients. Six contained cytoplasmic and eleven had perinuclear pattern. One patient with pauci-immune crescenteric glomerulonephritis had both cytoplasmic and perinuclear pattern and showed perinuclear pattern only on the follow-up test. All four patients with pauci-immune crescenteric glomerulonephritis and all three patients with polyarteritis nodosa revealed positive results. The titers of ANCA were significantly higher in the group with pauci-immune crescenteric glomerulonephritis, polyarteritis nodosa, and lupus nephritis (type 4) than in the other group of patients. Twelve of 45 patients exhibited crescent formation. Crescent was more frequently detected in patients with ANCA positive group (53%) than in negative group (11%), but there was no difference between cytoplasmic and perinuclear pattern. All two patients with ANCA positive IgA nephropathy revealed crescent formation, but only one out of four patients of IgA nephropathy with negative ANCA did. All four patients with reduction in titer levels showed improved renal function. Out of 2 patients without change in titers, one revealed no change and the other showed aggravated renal function. Conclusions: ANCA is a useful diagnostic marker in pauci-immune crescenteric glomerulonephritis and polyarteritis nodosa and can be found in lupus nephritis, IgA nephropathy, tubulointerstitial nephritis, and focal segmental glomerulosclerosis.