Intravenous immunoglobulins for steroid‐refractory esophageal involvement related to polymyositis and dermatomyositis: A series of 73 patients

Objective 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). Methods 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. Results 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. Conclusion 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.