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硬皮病(PSS)是一种病因尚不清楚的全性身疾病,预后与受累脏器相关。日本统计1970～1987年死亡患者24例,决定预后的脏器损害有:肺动脉高压6例,感染5例,肾损害4例,消化道障碍2例,心肌损害1例,肺出血1例,肺癌1例,不明4例。类固醇制剂对消除关节炎、肌炎和心包炎等症有效,而对皮肤硬化和脏器纤维化等固有病变疗效不佳。类固醇对预后没有直接影响,大量使用反而招致继发性感染。但最近报道类固醇冲击疗法治疗硬皮病并发的进行性肺动脉高压2例有效。硬皮病肾危象是伴有突发性、进展性高血压的急性肾功不全,预后极为不良。硬皮病并发的高血压极
Scleroderma (PSS) is an all-cause disease whose etiology is not known and whose prognosis is related to the affected organs. Japanese statistics from 1970 to 1987, 24 patients died, determine the prognosis of organ damage are: pulmonary hypertension in 6 cases, 5 cases of infection, 4 cases of renal damage, 2 cases of gastrointestinal disorders, myocardial damage in 1 case, 1 case of pulmonary hemorrhage, lung cancer 1 case, unknown 4 cases. Steroid preparations on the elimination of arthritis, myositis and pericarditis embolism effective, and skin lesions and organ fibrosis and other inherent lesions ineffective. Steroids have no direct effect on prognosis, but heavy use causes secondary infections. However, recently reported steroid shock therapy for the treatment of scleroderma complicated by progressive pulmonary hypertension in 2 cases. Scleroderma Kidney crisis is associated with acute, progressive hypertension in acute renal insufficiency, the prognosis is extremely poor. Scleroderma complicated by high blood pressure pole