Centre Hospitalier Universitaire Rouen, and INSERM U 905, University of Rouen IFRMP, Institute for Biochemical Research, Rouen, France.
Arthritis Care Res (Hoboken). 2010 Dec;62(12):1748-55. doi: 10.1002/acr.20325.
To assess the long-term outcome of esophageal complications in the group of patients receiving intravenous immunoglobulins (IVIG) for the treatment of severe steroid-refractory esophageal involvement related to polymyositis/dermatomyositis (PM/DM).
We retrospectively reviewed the medical records of 73 patients (39 with PM, 34 with DM) with steroid-resistant esophageal involvement. Esophageal involvement was evaluated by clinical and manometric investigations.
Seventy-three patients with steroid-refractory esophageal involvement related to PM/DM received IVIG therapy (2 gm/kg monthly). The median interval between PM/DM diagnosis and the onset of esophageal complications was 6 months. The most common clinical manifestations revealing esophageal dysfunction were dysphagia (69.9%), coughing while eating (61.6%), and gastroesophageal reflux into the pharynx and/or mouth (34.2%). Twenty-five patients exhibited life-threatening esophageal complications requiring exclusive enteral feeding; 33 patients (45.2%) with esophageal impairment developed aspiration pneumonia. Sixty patients (82.2%) exhibited resolution of esophageal clinical manifestations, leading to a return to normal oral feeding and ablation of feeding enteral tubes. Four other patients (5.5%) improved, although they still experienced mild dysphagia intermittently. Because of impaired cricopharyngeal muscle relaxation, another patient successfully underwent cricopharyngeal myotomy. Eight patients died from aspiration pneumonia (n=6) and cancer (n=2). Muscle weakness, thoracic myopathy, and aspiration pneumonia were independent predictive factors of IVIG-treated esophageal complications in PM/DM patients.
Our findings indicate that IVIG should be considered in life-threatening esophageal impairment complicating steroid-resistant PM/DM. We also suggest that combined therapy of IVIG and high-dose steroids may be the first-line therapy in PM/DM patients with life-threatening esophageal manifestations.
评估静脉注射免疫球蛋白（IVIG）治疗多发性肌炎/皮肌炎（PM/DM）相关严重激素抵抗性食管受累患者的食管并发症的长期预后。
我们回顾性分析了 73 例激素抵抗性食管受累患者（39 例 PM，34 例 DM）的病历。通过临床和测压检查评估食管受累情况。
73 例 PM/DM 相关激素抵抗性食管受累患者接受 IVIG 治疗（2 gm/kg 每月）。PM/DM 诊断与食管并发症发生之间的中位间隔为 6 个月。最常见的食管功能障碍临床表现为吞咽困难（69.9%）、进食时咳嗽（61.6%）和胃食管反流到咽部和/或口腔（34.2%）。25 例患者出现危及生命的食管并发症，需要专门的肠内喂养；33 例（45.2%）有食管损害的患者发生吸入性肺炎。60 例（82.2%）患者食管临床表现缓解，恢复正常口服喂养并拔除喂养肠内管。另有 4 例（5.5%）患者改善，尽管仍间歇性出现轻度吞咽困难。由于环咽肌松弛不良，另一名患者成功接受了环咽肌切开术。8 例患者因吸入性肺炎（n=6）和癌症（n=2）死亡。肌肉无力、胸肌病和吸入性肺炎是 PM/DM 患者 IVIG 治疗食管并发症的独立预测因素。
我们的研究结果表明，对于危及生命的激素抵抗性 PM/DM 合并食管受累，应考虑 IVIG 治疗。我们还建议，对于有危及生命的食管表现的 PM/DM 患者，IVIG 和大剂量类固醇联合治疗可能是一线治疗。
