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

Arthritis Care Res (Hoboken). 2010 Dec;62(12):1748-55. doi: 10.1002/acr.20325.


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.

Publication types

  • Comparative Study

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Dermatomyositis / complications
  • Dermatomyositis / drug therapy*
  • Drug Tolerance*
  • Esophageal Diseases / drug therapy*
  • Esophageal Diseases / epidemiology
  • Esophageal Diseases / etiology
  • Female
  • Follow-Up Studies
  • Glucocorticoids / pharmacology*
  • Humans
  • Immunoglobulins, Intravenous / administration & dosage
  • Immunoglobulins, Intravenous / therapeutic use*
  • Immunologic Factors / administration & dosage
  • Immunologic Factors / therapeutic use*
  • Male
  • Middle Aged
  • Polymyositis / complications
  • Polymyositis / drug therapy*
  • Prevalence
  • Retrospective Studies
  • Treatment Outcome
  • Young Adult


  • Glucocorticoids
  • Immunoglobulins, Intravenous
  • Immunologic Factors