Ferrarini M, Helfrich D J, Walker E R, Medsger T A, Whiteside T L
Department of Pathology, University of Pittsburgh School of Medicine, PA.
J Rheumatol. 1989 Jun;16(6):837-41.
In comparison to normal fibroblasts cultured in parallel, scleromyxedema fibroblasts grew less well, synthesized increased amounts of glycosaminoglycans (GAG) in vitro and had ultrastructural abnormalities. Serum obtained from a patient with scleromyxedema increased in vitro fibroblast proliferation but not the GAG synthesis per cell. Serum obtained after therapy, at the time when clinical improvement was observed, continued to stimulate fibroblast proliferation. Thus the serum factor stimulating the fibroblast proliferation did not modulate their GAG synthesis and had no direct relationship to disease activity.
与平行培养的正常成纤维细胞相比，硬皮病黏液水肿型成纤维细胞生长较差，在体外合成的糖胺聚糖（GAG）量增加，且存在超微结构异常。硬皮病黏液水肿型患者的血清在体外可增加成纤维细胞增殖，但不增加单个细胞的GAG合成。在观察到临床改善时，治疗后获得的血清继续刺激成纤维细胞增殖。因此，刺激成纤维细胞增殖的血清因子并未调节其GAG合成，且与疾病活动无直接关系。
