Belt E A, Kauppi M J, Kaarela K, Savolainen H A, Kautiainen H J, Lehto M U
Department of Orthopedics, Rheumatism Foundation Hospital, Heinola, Finland.
Clin Exp Rheumatol. 2000 Sep-Oct;18(5):601-4.
To describe arthritis mutilans (AM) deformity during the progression of rheumatic disease.
The development of mutilans-like hand deformities in 2 patients with juvenile chronic arthritis (JCA) and in 2 patients with adult onset rheumatoid arthritis (RA) are presented. The hands of these patients were evaluated at least at two time points during the course of disease using two different scoring methods based on differently summed Larsen grades of the hand joints.
Two patients (one with JCA and one with RA) showed AM changes after a disease period of less than 10 years and 2 not until after 30 years. The patients with adult onset disease were young at the onset of joint disease. Early wrist fusions were performed on both patients showing a slow development rate.
The development rate of AM is very variable, even in patients with the same diagnoses. Wrist fusion prevents shortening of the carpus and may decrease the development rate of AM.
描述风湿性疾病进展过程中的毁形性关节炎(AM)畸形。
介绍了2例青少年慢性关节炎(JCA)患者和2例成年发病类风湿关节炎(RA)患者中出现的类似毁形的手部畸形情况。在疾病过程中,至少在两个时间点使用基于手部关节不同总和的Larsen分级的两种不同评分方法对这些患者的手部进行评估。
2例患者(1例JCA患者和1例RA患者)在病程不到10年后出现AM改变,另外2例直到30年后才出现。成年发病的患者在关节疾病发病时较年轻。对2例显示进展缓慢的患者均进行了早期腕关节融合术。
即使在诊断相同的患者中,AM的发展速度也非常多变。腕关节融合可防止腕骨缩短,并可能降低AM的发展速度。