Rosenthal M
Helv Paediatr Acta. 1978 Aug;33(3):251-8.
Five children with Sharp syndrome are described presenting a non-erosive polyarthritis, hand and finger swelling, Raynaud phenomenon, myositis, dermatomyositis or SLE-like rash. Characteristic laboratory findings are, apart from elevated sedimentation rate, anemia and leucopenia, high titer IgM rheumatoid factors and antinuclear antibodies (ANA). The latter show speckled pattern, contain IgG, bind complement components and are directed against ribonuclease-sensitive nuclear antigens. All patients have antibodies against the so-called extractable nuclear antigens (Anti-ENA) and antibodies against ribonucleoproteins (Anti-RNP). Since children with Sharp syndrome rarely show renal or cerebral involvement, the prognosis seems to be fairly good.
本文描述了5例夏普综合征患儿，他们表现为非侵蚀性多关节炎、手部和手指肿胀、雷诺现象、肌炎、皮肌炎或系统性红斑狼疮样皮疹。除血沉升高、贫血和白细胞减少外，特征性实验室检查结果为高滴度IgM类风湿因子和抗核抗体（ANA）。后者呈斑点状模式，含有IgG，结合补体成分，并针对核糖核酸酶敏感的核抗原。所有患者均有针对所谓可提取核抗原（抗ENA）的抗体和针对核糖核蛋白（抗RNP）的抗体。由于夏普综合征患儿很少出现肾脏或脑部受累，预后似乎相当良好。
