Rigamonti C, Shand L M, Feudjo M, Bunn C C, Black C M, Denton C P, Burroughs A K
Liver Transplantation and Hepatobiliary Medicine, Royal Free Hospital, Pond St, Hampstead, London NW3 2QG, UK.
Gut. 2006 Mar;55(3):388-94. doi: 10.1136/gut.2005.075002. Epub 2005 Sep 8.
To evaluate the prognosis of primary biliary cirrhosis (PBC) together with systemic sclerosis (SSc), as this is unknown.
A PBC database of 580 patients identified 43 with PBC and SSc: two patients with PBC alone were matched to each PBC-SSc patient for serum bilirubin concentration at the initial visit. Forty (93%) patients had limited cutaneous SSc. At diagnosis of PBC, median values were: 49.7 years, bilirubin 17 micromol/l, and albumin 40.5 g/l. Liver diagnosis occurred a median 4.9 years after SSc in 24 (56%) patients. In matched patients, median values at diagnosis were: 53.2 years, bilirubin 12 micromol/l, and albumin 41 g/l. Median follow up was similar: 3.16 years (PBC-SSc) and 4.8 years (PBC alone). The risk of transplantation or death from diagnosis, adjusting for sex, age, log bilirubin, and alkaline phosphatase was significantly lower in PBC-SSc (hazard ratio 0.116, p=0.01) due to less transplantation (hazard ratio 0.068, p=0.006). The rate of bilirubin increase was less in PBC-SSc (p=0.04). Overall survival was similar (hazard ratio 1.11, p=0.948); there were nine deaths (21%) in PBC-SSc (six SSc related and two liver related) and nine (11%) in PBC alone (six liver related).
Liver disease has a slower progression in PBC-SSc compared with matched patients with PBC alone.
原发性胆汁性肝硬化（PBC）合并系统性硬化症（SSc）的预后尚不清楚，本研究旨在评估其预后情况。
在一个包含580例患者的PBC数据库中，识别出43例PBC合并SSc患者：为每例PBC-SSc患者匹配2例初诊时血清胆红素浓度相近的单纯PBC患者。40例（93%）患者为局限性皮肤型SSc。PBC诊断时，患者的中位年龄为49.7岁，胆红素水平为17 μmol/L，白蛋白水平为40.5 g/L。24例（56%）患者在SSc诊断后中位4.9年出现肝脏病变。在匹配患者中，诊断时的中位年龄为53.2岁，胆红素水平为12 μmol/L，白蛋白水平为41 g/L。中位随访时间相似：PBC-SSc组为3.16年，单纯PBC组为4.8年。校正性别、年龄、胆红素对数和碱性磷酸酶后，PBC-SSc患者从诊断开始的移植或死亡风险显著较低（风险比0.116，p = 0.01），原因是移植较少（风险比0.068，p = 0.006）。PBC-SSc患者胆红素升高率较低（p = 0.04）。总体生存率相似（风险比1.11，p = 0.948）；PBC-SSc组有9例死亡（21%）（6例与SSc相关，2例与肝脏相关），单纯PBC组有9例死亡（11%）（6例与肝脏相关）。
与匹配的单纯PBC患者相比，PBC-SSc患者的肝脏疾病进展较慢。
