Department of Internal Medicine, CHU Rouen, 76031 Rouen Cedex, France.
Br J Dermatol. 2010 Feb 1;162(2):337-44. doi: 10.1111/j.1365-2133.2009.09484.x. Epub 2009 Aug 29.
To date, no series has analysed long-term outcome in patients with polymyositis/dermatomyositis (PM/DM) with anti-PM-Scl antibody.
The aims of the present study were: (i) to assess clinical features and long-term outcome, including organ complications, functional course and mortality rate, in patients with isolated PM/DM with anti-PM-Scl antibody; and (ii) to evaluate prevalence, characteristics and long-term outcome of interstitial lung disease (ILD) in patients with isolated PM/DM with anti-PM-Scl antibody.
The medical records of 20 consecutive patients with isolated PM/DM with anti-PM-Scl antibody were reviewed.
Two patients (10%) achieved remission of PM/DM, whereas 14 (70%) improved and four (20%) had a worsened clinical status. Short-term recurrences (during tapering of therapy) occurred in nine patients and long-term recurrences (after discontinuation of therapy) in three patients. Moreover, patients with PM/DM with anti-PM-Scl antibody exhibited severe complications, as follows: oesophageal involvement (n = 4) requiring enteral feeding in three cases, ventilatory insufficiency (n = 3) requiring mechanical ventilation in two cases; three other patients had cancer. Interestingly, patients with PM/DM with anti-PM-Scl antibody often presented symptoms that are usually found in antisynthetase syndrome, i.e. hyperkeratotic rhagadiform hand symptoms (n = 2; 10%), Raynaud's phenomenon (n = 8; 40%), arthralgia/arthritis (n = 7; 35%) and ILD (n = 12; 60%). In our cohort, the associated ILD often required combined therapy of steroids and immunosuppressive agents.
Our series suggests that the presence of anti-PM-Scl antibody is not a good prognostic factor in patients with PM/DM, as there appears to be an association with lung and oesophageal involvement; in addition, anti-PM-Scl antibody may coexist with malignancy in patients with PM/DM. Furthermore, anti-PM-Scl antibody-positive patients with PM/DM often exhibit 'mechanic's hands', Raynaud's phenomenon and joint involvement. Our latter findings raise the possibility that the immunogenetic background influences the autoantibody status of these patients; HLA-DR3 has, in fact, been found in association with antisynthetase syndrome antibodies and with anti-PM-Scl antibodies.
迄今为止，尚无研究分析抗 PM-Scl 抗体阳性的多发性肌炎/皮肌炎（PM/DM）患者的长期预后。
本研究旨在：（i）评估抗 PM-Scl 抗体阳性的单纯 PM/DM 患者的临床特征和长期预后，包括器官并发症、功能病程和死亡率；（ii）评估抗 PM-Scl 抗体阳性的单纯 PM/DM 患者间质性肺病（ILD）的发生率、特征和长期预后。
回顾性分析 20 例连续的抗 PM-Scl 抗体阳性的单纯 PM/DM 患者的病历。
2 例（10%）患者 PM/DM 缓解，14 例（70%）患者改善，4 例（20%）患者病情恶化。9 例患者在减药过程中出现短期复发，3 例患者停药后出现长期复发。此外，抗 PM-Scl 抗体阳性的 PM/DM 患者出现严重并发症，如下：食管受累（n=4），3 例需肠内喂养；呼吸衰竭（n=3），2 例需机械通气；另外 3 例患者患有癌症。有趣的是，抗 PM-Scl 抗体阳性的 PM/DM 患者常出现抗合成酶综合征的症状，即角化过度的裂手症状（n=2；10%）、雷诺现象（n=8；40%）、关节痛/关节炎（n=7；35%）和 ILD（n=12；60%）。在我们的队列中，ILD 常需要联合使用皮质类固醇和免疫抑制剂治疗。
本研究提示，抗 PM-Scl 抗体在 PM/DM 患者中并非良好的预后因素，因为它似乎与肺和食管受累有关；此外，抗 PM-Scl 抗体可能与 PM/DM 患者的恶性肿瘤共存。此外，抗 PM-Scl 抗体阳性的 PM/DM 患者常出现“技工手”、雷诺现象和关节受累。我们的研究结果提示，免疫遗传背景可能影响这些患者的自身抗体状态；事实上，HLA-DR3 与抗合成酶综合征抗体和抗 PM-Scl 抗体有关。
