Jeffrey R F, Khan A A, Davison A, Aparicio S, Gooi H C, Will E J, Davison A M
Department of Renal Medicine, St. James's University Hospital, Leeds.
Br J Clin Pract. 1994 Jul-Aug;48(4):180-4.
We have reviewed 39 adult patients who presented over a 5-year period with biopsy confirmed renal disease in association with positive antineutrophil cytoplasmic antibody (18 with C-ANCA, 21 with P-ANCA). Twenty-three (59%) had primary systemic vasculitis, typically with aggressive renal histology including focal necrotising and crescentic glomerulonephritis. In the remaining patients a wide range of clinical syndromes and renal histological appearances were apparent: 30 had abnormal renal function (serum creatinine > 140 mumol/l), including 17 who were initially dialysis-dependent. Intensive immunosuppression was administered in 33 cases. When response was assessed at 3 months, renal function was stable or improved in 17 (52%), 5 of whom were able to discontinue dialysis. There was, however, an appreciable early mortality and, at latest follow-up (1-57 months), 12 patients had died and 8 were on the dialysis programme. On immunosuppression, ANCA became negative in the majority (median time 1.5 months) but recurred during clinical relapse in 11 cases. In asymptomatic patients (12 cases), the reappearance of ANCA positivity did not herald clinical relapse. The ANCA assay has increased awareness of systemic vasculitis but not removed the need for histological confirmation before instituting immunosuppression.
我们回顾了39例成年患者，这些患者在5年期间因活检证实患有肾脏疾病且抗中性粒细胞胞浆抗体呈阳性而就诊（18例为C-ANCA阳性，21例为P-ANCA阳性）。23例（59%）患有原发性系统性血管炎，典型表现为侵袭性肾脏组织学改变，包括局灶性坏死性和新月体性肾小球肾炎。其余患者表现出广泛的临床综合征和肾脏组织学表现：30例肾功能异常（血清肌酐>140μmol/L），其中17例最初依赖透析。33例患者接受了强化免疫抑制治疗。在3个月时评估反应时，17例（52%）患者的肾功能稳定或改善，其中5例能够停止透析。然而，早期死亡率相当可观，在最近一次随访（1 - 57个月）时，12例患者死亡，8例仍在透析治疗。在免疫抑制治疗过程中，大多数患者的ANCA转为阴性（中位时间为1.5个月），但11例患者在临床复发时ANCA再次出现。在无症状患者（12例）中，ANCA阳性再现并未预示临床复发。ANCA检测提高了对系统性血管炎的认识，但在开始免疫抑制治疗之前，仍需要组织学证实。
