环磷酰胺脉冲治疗小儿系统性硬化症

Ryumachi. [Rheumatism] Pub Date : 2003-10-01
Naomi Iwata, Takako Miyamae, Tomoyuki Imagawa, Shigeki Katakura, Masaaki Mori, Yokoh Aihara, Shumpei Yokota, Megumi Kondo, Tetsuo Sasaki, Yoshiro Ikezawa
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引用次数: 0

摘要

我们遇到了3例小儿系统性硬化症患者。患者1在10岁时有系统性硬皮病、色素沉着和间质性肺炎。发病9个月后给予静脉环磷酰胺脉冲治疗作为诱导治疗。初步治疗后,间质性肺炎、硬皮病、皮肤总评分等临床表现明显改善。患者2为7岁女童,主诉系统性硬皮病和色素沉着,并发肺动脉高压。发病6个月后给予静脉环磷酰胺脉冲治疗。她的硬皮病和总皮肤评分得到改善,肺动脉高压没有恶化。三号病人是一名15岁的女孩。她最初的治疗是单独服用维生素E。她在发病两年半后住进我院。尽管给予包括环磷酰胺在内的免疫抑制治疗,但严重的病情持续存在,并于5个月后死亡。由于环磷酰胺脉冲治疗在病程早期,患者1和2有可能实现并保持临床表现的显着改善。我们进一步观察到他们的皮肤总评分下降,而临床表现改善。
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[Cyclophosphamide pulse therapy for pediatric systemic sclerosis].

We encountered three patients with pediatric systemic sclerosis. Patient 1 had systemic scleroderma, pigmentation and interstitial pneumonia at the age of 10 years. Nine months after disease onset, she was treated with intravenous cyclophosphamide pulse therapy as induction therapy. After the initial treatment, the following clinical manifestations were dramatically improved: interstitial pneumonia, scleroderma and total skin score. Patient 2 was a 7-year-old girl, who complained of systemic scleroderma and pigmentation, and was found to have pulmonary hypertension. Six months after disease onset, she was administrated intravenous cyclophosphamide pulse therapy. Her scleroderma and total skin score were improved and the pulmonary hypertension did not deteriorate. Patient 3 was a 15-year-old girl. Her initial treatment was vitamin E alone. She was admitted to our hospital two and half years after disease onset. Although she was given immunosuppressive therapy including cyclophosphamide, the severe condition of persisted, and she died after five months. It became possible for patient 1 and 2 to achieve and maintain a marked improvement of the clinical manifestations as a result of cyclophosphamide pulse therapy early in the course of the disease. We further observed that their total skin score was decreasing while the clinical manifestations improved.

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