Englert H, Kirkham S, Moore J, Poon T S, Katelaris C, McGill N, Schrieber L, Manolios N
Department of Rheumatology, Westmead Hospital, Sydney, New South Wales, Australia.
Intern Med J. 2008 Sep;38(9):692-6. doi: 10.1111/j.1445-5994.2007.01593.x. Epub 2008 Mar 7.
The aim of the study was to assess the structural and functional effects of autologous stem cell transplantation (ASCT) on scleroderma finger clawing.
Using photocopies of hands of five scleroderma patients who underwent ASCT using photocopies of hands. Functional assessments used a standardized questionnaire.
Pre-ASCT, synovitis and tenosynovitis were present in five and four patients, respectively. Modified Rodnan hand skin scores ranged from 6-12/12. Following pulsed chemotherapy, synovitis resolved. Tenosynovitis often did not. Post-ASCT, skin scores fell in four patients (range 0-6/12). Hand tenosynovitis resolved. With disease remission hand function globally improved. Functional improvement, noted early (+3 months) and continuously (+12 months) in disease remitters, occurred in all areas of function. Greatest hand-functional improvement related to paid employment, followed by self-care and hygiene, home-care activities and least by hobbies/sports. The second to fifth metacarpophalangeal width was reproducible and independent of ASCT therapy. In contrast, hand length and measures of abducted finger span (first to fifth fingertip and second to fifth fingertip distance) improved. Finger abduction (abducted first to fifth fingertips/second to fifth metacarpophalangeal width) was a more sensitive discriminator of finger clawing than hand length or hand length/second to fifth metacarpophalangeal width.
ASCT improved hand scleroderma over 12 months and resolved previously refractory tenosynovitis. ASCT was unnecessary to treat scleroderma synovitis. ASCT secondarily improved hand function (paid employment, followed by self-care, home care, then by sport/hobbies). Loss of finger abduction was a more sensitive measure of finger clawing than apparent loss of hand length.
本研究旨在评估自体干细胞移植（ASCT）对硬皮病手指挛缩的结构和功能影响。
使用五名接受ASCT的硬皮病患者手部的影印件。功能评估采用标准化问卷。
ASCT前，分别有5例和4例患者存在滑膜炎和腱鞘炎。改良Rodnan手部皮肤评分范围为6 - 12/12。脉冲化疗后，滑膜炎消退。腱鞘炎通常未消退。ASCT后，4例患者的皮肤评分下降（范围0 - 6/12）。手部腱鞘炎消退。随着疾病缓解，手部功能总体改善。疾病缓解者在早期（+3个月）和持续（+12个月）出现功能改善，所有功能领域均如此。手部功能改善最大的方面与有偿工作相关，其次是自我护理和卫生、家庭护理活动，而与爱好/运动相关的改善最小。第二至第五掌指关节宽度具有可重复性且与ASCT治疗无关。相比之下，手长和外展手指跨度（第一至第五指尖和第二至第五指尖距离）有所改善。手指外展（第一至第五指尖外展/第二至第五掌指关节宽度）比手长或手长/第二至第五掌指关节宽度更能敏感地鉴别手指挛缩。
ASCT在12个月内改善了手部硬皮病并解决了先前难治的腱鞘炎。治疗硬皮病滑膜炎无需进行ASCT。ASCT继而改善了手部功能（有偿工作，其次是自我护理、家庭护理，然后是运动/爱好）。手指外展丧失比手长明显缩短更能敏感地衡量手指挛缩。
