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

Abstract

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.

Recovery from severe dysphagia in systemic sclerosis - myositis overlap: a case report

“…Marie et al reported a French multicenter series of 73 patients with PM and DM who received IVIG for steroid refractory oesophageal involvement 4 . Nine (12.3%) patients required feeding via a PEG or percutaneous endoscopic jejunostomy.…”

“…Patients' refractory to corticosteroids or who require high doses of corticosteroids as maintenance therapy, may respond to immunosuppressive agents such as methotrexate, azathioprine, cyclophosphamide, cyclosporine or mycophenolate mofetil 3 . Intravenous immunoglobulin (IVIG) and rituximab are effective in patients with severe, progressive or refractory inflammatory myopathy [4][5][6][7][8] . Patients with refractory oesophageal involvement require PEG feeding and 82.2% of patients may improve with repeated courses of IVIG…”

A Case of Refractory Dysphagia in the Context of Polymyositis Treated with Subcutaneous Immunoglobulin and Review of the Literature

“…Patients were treated with IVIg (2 g/kg over 2 days monthly for a mean of 7 months) and 60/73 (82%) patients showed a rapid and significant improvement in esophageal symptoms [13].…”

Immunoglobulin Treatment in Polymyositis and Dermatomyositis

“…A rapid onset of therapeutical effects was substantiated by Marie et al (2010) in a recent retrospective multicentre study reviewing 73 patients with oesophageal involvement in which intravenous immunoglobulin (1 g/kg daily for two consecutive days each month for at least seven months) produced improvement within two weeks after the first infusion with a clinical resolution or a marked improvement of oesophageal clinical involvement in 65 patients (89%), thus suggesting their use as first-line therapy in life-threatening oesophageal manifestations. This data confirms previous studies in smaller series (Marie et al, 1999;Cherin et al, 2001). Contrasting results have been documented in interstitial lung disease associated with polymyositis and dermatomyositis, in which only a small number cases show benefits from intravenous immunoglobulin (Suzuki et al, 2009).…”