Rau R
Schweiz Med Wochenschr. 1977 Sep 3;107(35):1235-7.
Besides lymphodenopathy and splenomegaly, hepatomegaly may also be detected in 25-50% of children with juvenile rheumatoid arthritis. This is particularly evident in patients with complete Still's syndrome. The hepatomegaly increases during relapse situations and disappears during remissions. Transient icterus, elevation of aminotransferases and delayed bromsulfalein excretion have been reported, particularly in patients with complete Still's syndrome, and indicate impairment of liver function. Liver biopsies have been performed only rarely and show nonspecific infiltrations of portal fields with lymphocytes and, in a few cases, "autoimmune" hepatitis and even cirrhosis with portal hypertension. Plasma cell hepatitis with affection of joints can be readily differentiated from juvenile rheumatoid arthritis: the synovitis is merely transiet and disappears with institution of steroid therapy. As in the adult, severe liver dysfunction leads to remission of arthritis. Amyloidosis should be considered in every case of long-lasting hepatomegaly.
除淋巴结病和脾肿大外，25%至50%的幼年类风湿性关节炎患儿也可能出现肝肿大。这在完全型斯蒂尔综合征患者中尤为明显。肝肿大在病情复发时加重，缓解期消失。有报道称，特别是在完全型斯蒂尔综合征患者中，会出现短暂性黄疸、转氨酶升高和溴磺酞排泄延迟，提示肝功能受损。肝活检很少进行，结果显示门静脉区有淋巴细胞非特异性浸润，少数病例出现“自身免疫性”肝炎，甚至伴有门静脉高压的肝硬化。关节受累的浆细胞性肝炎很容易与幼年类风湿性关节炎相鉴别：滑膜炎只是短暂性的，类固醇治疗后会消失。与成人一样，严重肝功能障碍会导致关节炎缓解。对于每例长期肝肿大患者都应考虑淀粉样变性。
