Denisiewicz-Rostropowicz K, Romicka H
Rev Rhum Mal Osteoartic. 1980 Feb;47(2):93-6.
The authors study the diagnosis and the prognosis in 25 children with systemic lupus erythematosus and in 22 children with dermatomyositis. The most frequent signs when the diagnosis is established are articular involvements, erythema of the face, renal disorders and the presence of LE cells. The symptoms are often misleading, which explains the long delay between the first sign of the disease and the time of diagnosis, which can vary between one month to nine years. The prognosis of this disease has improved because of modern treatments : only four patients died, three of them because of renal failure and one because of cerebral embolism, after six months, one year, three years and ten months, In dermatomyositis the most frequent signs at the time of diagnosis are skin involvements and muscular and articular symptoms. The period of time between the onset of the disease and the diagnosis is shorter : one month to four years, 19 times equal to or less than one year, because the clinical picture is quite clear. Ten times the clinical evolution lead to a complete cure, 9 times a clear improvement with sequelae was observed : retractile myelosclerosis, calcinosis and medullary compression. One patient died of intestinal ulceration and two of pneumonia aggravated by involvement of the respiratory muscles.
作者对25例系统性红斑狼疮患儿和22例皮肌炎患儿的诊断和预后进行了研究。确诊时最常见的体征为关节受累、面部红斑、肾脏疾病和狼疮细胞的出现。症状常常具有误导性，这就解释了从疾病的首发症状到确诊之间存在较长时间延迟的原因，延迟时间在1个月至9年之间不等。由于现代治疗方法，这种疾病的预后有所改善：仅有4例患者死亡，其中3例死于肾衰竭，1例死于脑栓塞，分别发生在6个月、1年、3年和10个月后。在皮肌炎中，确诊时最常见的体征为皮肤受累以及肌肉和关节症状。从疾病发作到确诊的时间较短：为1个月至4年，其中19次等于或少于1年，因为临床表现相当明确。临床进展有10次导致完全治愈，9次观察到有明显改善但伴有后遗症：缩窄性骨髓硬化、钙质沉着和髓质受压。1例患者死于肠道溃疡，2例死于因呼吸肌受累而加重的肺炎。
