全身性強皮症に関する最近の話題

抄録

Recent advances in understanding the etiology and treatment of systemic sclerosis (SSc) were overviewed. In vivo overexpression of connective tissue growth factor (CTGF) mRNA has been demonstrated in dermal fibroblasts in patients with SSc. Serum CTGF levels are elevated in SSc patients and are correlated with the extent of skin and lung fibrosis. Thus, CTGF appears to be the key molecule in the pathogenesis of the disease. Fetal microchimerism is another issue in the pathogenesis of SSc. Recent studies have demonstrated that fetal microchimerism was more frequent in women with SSc than in normal women. Additional investigations are needed to verify the role of microchimerism in the pathogenesis of the disease. The effects of low-dose and high-dose D-penicillamine (D-Pen) were compared in a double-blind, randomized, controlled study. High-dose D-Pen was not more effective than low-dose D-Pen, suggesting that D-Pen itself may not be effective at all. Photopheresis (extracorporeal photochemotherapy) may have limited effects, but the results of a recent crossover trial were disappointing. Low-dose relaxin was effective in a double-blind, placebocontrolled study, but high-dose relaxin was not more effective than placebo. Epoprostenol (prostaglandin I₂) seems to be an effective agent for treating isolated pulmonary hypertension in patients with SSc.