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.


CONCLUSIONS
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.
Marie
I
I
Centre Hospitalier Universitaire Rouen, and INSERM U 905, University of Rouen IFRMP, Institute for Biochemical Research, Rouen, France. isabelle.marie@chu-rouen.fr
Menard
J-F
JF
Hatron
P Y
PY
Hachulla
E
E
Mouthon
L
L
Tiev
K
K
Ducrotte
P
P
Cherin
P
P
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003882:Dermatomyositis / Q000150:complications / Q000188:drug therapy*; D004361:Drug Tolerance; D004935:Esophageal Diseases / Q000188:drug therapy* / Q000453:epidemiology / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000494:pharmacology*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage / Q000627:therapeutic use*; D007155:Immunologic Factors / Q000008:administration & dosage / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D017285:Polymyositis / Q000150:complications / Q000188:drug therapy*; D015995:Prevalence; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors