Pathologic features of a familial arthropathy associated with congenital flexion contractures of fingers.
Pathologic features of a familial arthropathy associated with congenital flexion contractures of fingers.
复制标题
与先天性手指屈曲挛缩相关的家族性关节病的病理特征。
作者：
B. Athreya;B. Athreya;H. Schumacher
对三个最近被确认为家族性关节病的兄弟姐妹进行了临床研究，并对滑膜进行了光学和电子显微镜检查。光镜下，3例滑膜均表现为滑膜增生、坏死绒毛、嗜酸性粒细胞和PAS阳性物质沉积，大量多核巨细胞。1例滑膜电子显微镜下可见巨细胞，内有大量粗面光滑的内质网。一些吞噬细胞在空泡或凹陷中含有变性的胶原。滑膜表面可见颗粒状和纤维素样物质，未见淀粉样物。这种对称性关节病目前在三个家庭中报道，似乎具有滑膜的组织学特征，这使得它与其他已知的关节疾病截然不同。
Three siblings with a recently recognized form of familial arthropathy were studied clinically and by light and electron microscopy of the synovium. On light microscopy, the synovium from all 3 children showed synovial hyperplasia, necrotic villi, deposition of eosinophilic and PAS-positive material, and large numbers of multinucleated giant cells. Electron microscopy of the synovium in one patient revealed giant cells with profuse rough and smooth endoplasmic reticulum. Some phagocytic cells contained degenerated collagen in vacuoles or invaginations. There was granular and fibrinlike material on the synovial surface, but no amyloid. This symmetrical arthropathy now reported in three families appears to have characteristic histological appearance of synovium that allows its identification as distinct from other known joint diseases.