Stoeber E
Eur J Pediatr. 1981 Feb;135(3):225-8. doi: 10.1007/BF00442095.
A total of 433 children, hospitalized in the Rheumatic Children's Hospital Garmisch-Partenkirchen, were followed on average for 15 years (range 10-22 years) after the onset of definite juvenile chronic arthritis. This paper reveals clearly that the different subgroups of chronic rheumatic disease in childhood with their different complications have a definite relationship to prognosis. Severe limitation with inability to attend normal school or employment occurred in 13.4% of children with systemic polyarticular arthritis (Still's syndrome) and 11.4% of the non-systemic cases, but not in the pauci-articular group. In the latter group 82.5% of the children remained without disability or with only slight impairment: this is significantly better than in the systemic or non-systemic polyarticular groups. Although the high incidence of chronic rheumatic iridocyclitis is common in children with pauci-articular arthritis, none of this group had been handicapped by severe eye complications or blindness, in contrast to several early cases with systemic or non-systemic polyarthritis. This might be due to regular eye checks in the pauci-articular group. Of the children in the systemic polyarticular group 10% were dwarfed. Mortality in the systemic group was 13.8%, in contrast to 1% in the non-systemic polyarticular and 0% in the pauci-articular arthritis group. Secondary amyloidosis was the most important cause of death, mainly in systemic cases. Of the children whose amyloidosis had been verified, 44% died in their second or third decades, mostly with uraemia.
共有433名在加米施-帕滕基兴风湿儿童医院住院的儿童,在确诊为幼年慢性关节炎后平均随访了15年(范围为10至22年)。本文清楚地表明,儿童慢性风湿性疾病的不同亚组及其不同并发症与预后有明确关系。全身性多关节型关节炎(斯蒂尔综合征)患儿中有13.4%、非全身性病例中有11.4%出现严重功能受限,无法正常上学或就业,但少关节型组中未出现这种情况。在后一组中,82.5%的儿童没有残疾或仅有轻微损伤:这明显优于全身性或非全身性多关节型组。虽然慢性风湿性虹膜睫状体炎在少关节型关节炎患儿中发病率很高,但与一些全身性或非全身性多关节炎的早期病例不同,该组中没有患儿因严重眼部并发症或失明而致残。这可能是由于少关节型组进行了定期眼部检查。全身性多关节型组中有10%的儿童身材矮小。全身性组的死亡率为13.8%,相比之下,非全身性多关节型组为1%,少关节型关节炎组为0%。继发性淀粉样变性是最重要的死亡原因,主要发生在全身性病例中。在已证实患有淀粉样变性的儿童中,44%在二三十岁时死亡,大多死于尿毒症。