Department of Medicine, Mitsui Memorial Hospital, Kanda-Izumi-cho, Chiyoda-ku, Tokyo, Japan.
Clin Exp Nephrol. 2010 Aug;14(4):325-32. doi: 10.1007/s10157-010-0278-z. Epub 2010 Mar 26.
Several cases of rheumatoid arthritis (RA) with myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA)-associated crescentic glomerulonephritis (CrGN) have been reported. However, its clinical characteristics are not clear.
We summarized 3 patients of concurrent RA and MPO-ANCA-associated CrGN, diagnosed in our hospital from 1992 to 2006, and compared their clinicopathological data with those of 10 MPO-ANCA-associated CrGN patients without RA in the same period.
All three RA patients were middle-aged or young adult women with 7-14 years of RA history. The initial clinical symptom was microhematuria, and mean duration from hematuria onset to histological confirmation of CrGN was 17 months. At renal biopsy, serum creatinine concentration (sCr) was modestly elevated, with the mean value of 3.4 mg/dl. Crescents were detected in 30% of glomeruli, whereas advanced glomerular sclerosis, tubular atrophy, and interstitial fibrosis were also observed. In comparison with patients without RA, patients with RA were significantly younger and showed a longer duration from the onset to histological confirmation of CrGN. Serum creatinine concentration at referral was significantly lower; however, estimated glomerular filtration rate (eGFR) was comparable. The Birmingham Vasculitis Activity Score and the Disease Extent Index were significantly lower, and pathological examination showed less crescent formation and a tendency to advanced glomerular sclerosis in patients with RA.
In patients with RA, MPO-ANCA-associated CrGN appeared to develop at younger ages and often showed a slowly progressive deterioration of the renal function with slight extrarenal manifestations. These smoldering clinical features may result in late referral from rheumatologists to nephrologists and therefore poor prognosis.
已有数例类风湿关节炎(RA)合并髓过氧化物酶-抗中性粒细胞胞质抗体(MPO-ANCA)相关新月体肾炎(CrGN)的报道。然而,其临床特征尚不清楚。
我们总结了我院 1992 年至 2006 年间诊断的 3 例同时患有 RA 和 MPO-ANCA 相关 CrGN 的患者,并将其临床病理资料与同期 10 例无 RA 的 MPO-ANCA 相关 CrGN 患者进行比较。
3 例 RA 患者均为中年或青年女性,RA 病史 7-14 年。首发症状为镜下血尿,血尿出现至组织学确诊 CrGN 的平均时间为 17 个月。肾活检时血清肌酐浓度(sCr)轻度升高,平均为 3.4mg/dl。肾小球中新月体占 30%,同时还观察到晚期肾小球硬化、肾小管萎缩和间质纤维化。与无 RA 患者相比,RA 患者年龄明显更小,且从发病到组织学确诊 CrGN 的时间更长。就诊时血清肌酐浓度显著较低;然而,估算肾小球滤过率(eGFR)相当。伯明翰血管炎活动评分和疾病程度指数显著较低,病理检查显示 RA 患者新月体形成较少,且晚期肾小球硬化趋势更为明显。
在 RA 患者中,MPO-ANCA 相关 CrGN 似乎发病年龄更小,且肾功能进行性恶化往往较为缓慢,伴有轻微的肾外表现。这些隐匿的临床特征可能导致风湿科医生较晚转诊给肾病科医生,从而导致预后不良。