41例抗肾小球基底膜抗体相关疾病的临床和病理分析

目的了解抗肾小球基底膜（GBM）抗体相关疾病的不同临床类型及其临床病理特点。方法对我科近6年来检测出的41例抗GBM抗体相关疾病的临床病理资料进行回顾性分析。结果22/41例为Goodpasture病（GP），其中2例肾功能正常。32/41例患者为单纯抗GBM抗体阳性，其中31/32例男性，平均年龄（26.8±9.7）岁。9/41例抗GBM抗体伴抗中性粒细胞胞浆抗体（ANCA）阳性，其中7/9例为女性，平均年龄（44.5±19.6）岁。两组在性别和发病年龄上差异有显著性（P＜0.05）。32/41例作了肾活检，平均发病至肾活检时间为（62.7±43.5）d。2/32例肾功能正常的GP患者为轻度系膜增生性肾炎。30/32例为新月体肾炎，其中24/30例（80%）患者的肾小球100%受累，多伴有毛细血管襻和球囊严重破坏。免疫荧光检查仅16/23例呈典型的IgG、C3沿肾小球毛细血管襻（GCW）呈线样沉积;7/23例表现为IgG和（或）C3等沿GCW呈细颗粒状沉积，少数伴有系膜区沉积。典型和不典型的免疫荧光改变与光镜病理的严重程度不相关（P＞0.05）。所有患者均有贫血、血尿和蛋白尿，其中7/41例表现为肾病综合征。经强化免疫抑制治疗，4例患者临床完全缓解或好转，包括2例轻度系膜增生性肾炎的GP患者。其余患者均依赖肾脏替代疗法或死亡。结论中国人抗GBM抗体相关疾病并不少见。单纯抗GBM抗体组多发于青年男性，双抗体阳性组多发于中老年女性。抗GBM抗体相关疾病多预后差，肾脏病理多为新月体性肾炎，但免疫病理并非均表现为典型的IgG、C3沿GCW呈线条样沉积。仅少数无少尿的轻型患者或肾功能损伤轻者可临床完全缓解或好转。

A clinical and pathological analysis of 41 patients with anti-glomerular basement membrane antibody related diseases

OBJECTIVE: To investigate the clinical and pathological characteristics of anti-glomerular basement membrane (GBM) antibody associated diseases with different clinical patterns. METHODS: The clinical and pathological data of 41 patients with anti-GBM antibody associated diseases, screened in our institute in recent six years, were retrospectively analyzed. RESULTS: 22 of the 41 patients presented as Goodpasture syndrome, 2 of them having normal renal function. 32 of the 41 patients had anti-GBM antibody positive only (single positive). 31 of the 32 single positive patients were male with an average age of (26.8 +/- 9.7) years. The remaining 9 patients were both anti-GBM antibody and antineutrophil cytoplasmic antibody (ANCA) positive (double positive); 7 of the 9 were female with an average age of (44.5 +/- 19.6) years. There was significant difference in gender and age between the single and double positive groups (P < 0.05). 30 of the 32 patients with renal biopsy had crescentic glomerulonephritis and the remaining two with normal renal function had mild mesangial proliferative glomerulonephritis only. 24 of the 30 (80%) crescentic glomerulonephritis patients had all glomeruli affected by crescents with severe glomerular capillary loop and Bowman's capsule damage. With immunofluorescence examination, 16 of the 23 cases had typical IgG and C(3) linear deposition along glomerular capillary walls, but the remaining 7 had fine granular IgG and/or C(3) deposition. In a few cases, granular deposition was found even in mesangium. There was no correlation between the immunofluorescence patterns and the severity of glomerular damage (P > 0.05). All patients had anemia, hematuria and proteinuria, 7 of the 41 cases presented as nephrotic syndrome. After intensive immunosuppressive therapy, only four patients, including the two patients with normal renal functions, achieved remission, the remaining patients either were in renal replacement therapy or died. CONCLUSIONS: Anti-GBM antibody associated diseases are not rare in China. Single positive patients are mainly young male, while double positive patients are mainly middle-aged female. Most of the patients with anti-GBM antibody associated diseases have crescentic glomerulonephritis (rapidly progressive glomerulonephritis, RPGN) with a bad prognosis. However, not all the patients have typical linear deposition of IgG and C(3) along glomerular capillary walls. Only a few non-oliguric patients with normal renal function or mild renal damage may achieve remission.