García Consuegra J, Antelo M C, Torreblanca J
Servicio de Preescolares, Hospital Infantil La Paz, Madrid.
An Esp Pediatr. 1987 Nov;27(5):362-6.
Eight cases of dermatomyositis and one of polymyositis were studies among five male and four female patients. All had criteria definitive of the disease. All nine patients were give prednisone; six of them responded favorably to treatment and after discontinuing still remain asymptomatic. Three male patients have had a worse response to therapy, two of them deceased and the third still remains symptomatic, but very slowly improving after fourteen months with steroids and cyclophosphamide. These three patients have had chronic cutaneous vasculitic ulcers and this is a sign of bad prognosis. Both deceased patients exhibited important visceral complications (digestive and pulmonary). A more aggressive therapy has been suggested in these forms of worse clinical evolutions, including use of immunosuppressors.
对8例皮肌炎患者和1例多发性肌炎患者进行了研究,其中5例男性患者,4例女性患者。所有患者均符合该疾病的确切诊断标准。所有9例患者均接受了泼尼松治疗;其中6例对治疗反应良好,停药后仍无症状。3例男性患者对治疗反应较差,其中2例死亡,第3例仍有症状,但在接受类固醇和环磷酰胺治疗14个月后症状正在缓慢改善。这3例患者患有慢性皮肤血管炎性溃疡,这是预后不良的迹象。两名死亡患者均出现了重要的内脏并发症(消化系统和肺部)。对于这些临床进展较差的病例,建议采用更积极的治疗方法,包括使用免疫抑制剂。