Division of Rheumatology, Department of Internal Medicine, University of Florence, Florence, Italy.
Clin Exp Rheumatol. 2013 Mar-Apr;31(2 Suppl 76):181-3. Epub 2013 Jul 23.
Stiff skin syndrome (SSS) is a rare scleroderma-like syndrome characterised by stone hard skin, joint limitation and progressive restriction of chest that may lead to death. We describe the efficacy of haematopoietic autologous stem cell transplantation (HSCT) in a case of SSS secondary to a smouldering myeloma (SM), with severe joint disability, lung interstitial disease and oesophageal dysfunction. The patient was evaluated at 1, 12 and 18 months after HSCT, clinically (joint motility, HAQ and NYHA for dyspnoea) and instrumentally (DLCO, chest HRCT, oesophagus x-ray). After 18 months since HSCT, we observed a high improvement, contemporaneously to SM remission, of HAQ, joint motility, lung (at DLCO and HRCT) and oesophageal abnormalities.
硬皮病综合征（SSS）是一种罕见的类硬皮病综合征，其特征为皮肤如石般坚硬、关节受限以及胸部进行性受限，可能导致死亡。我们描述了造血自体干细胞移植（HSCT）在继发于冒烟型骨髓瘤（SM）的 SSS 中的疗效，该患者存在严重的关节残疾、肺间质疾病和食管功能障碍。在 HSCT 后 1、12 和 18 个月对患者进行了评估，包括临床（关节运动、HAQ 和呼吸困难的 NYHA 分级）和仪器检查（DLCO、胸部 HRCT、食管 X 线）。HSCT 后 18 个月，我们观察到 SM 缓解的同时，HAQ、关节运动、肺部（在 DLCO 和 HRCT 上）和食管异常都有较高的改善。
