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

Abstract

Polymyositis (PM) is a systemic inflammatory disease affecting muscles and several other organs, including gastrointestinal system, in which the main symptom is dysphagia.Here, we describe a case of a 57-years-old woman presenting a difficult-to-manage dysphagia related to polymyositis, treated with benefit with Subcutaneous Immunoglobulins (SCIg). Beyond symptomatic therapies, immunosuppressive therapies (glucocorticoids, methotrexate, azathioprine, mycophenolatemofetil, and cyclosporine) are the main treatment of PM related dysphagia. Nowadays, few data are available to evaluate effectiveness and safety of intravenous immunoglobulins on esophageal involvement in refractory PM and only anecdotal cases are reported about the use of SCIg in the treatment of dysphagia in inflammatory myopathies (including inclusion body myositis). Up to date, this is the second observation describing an improvement of dysphagia in a subject affected by PM treated with SCIg; further studies are needed to evaluate the effectiveness of SCIg in the management of these patients.

Impact and Management of Dysphagia in Inflammatory Myopathies

“…In steroid-refractory cases and if immunosuppressive agents also fail or are not tolerated, intravenous immunoglobulins (IVIg) have shown to be effective in the treatment of myositis-associated dysphagia and esophageal involvement in patients with PM or DM [5,66,67]. More recently, the use of subcutaneous immunoglobulins (SCIg) has been demonstrated to be positive in two case reports with dysphagia in PM [68,69].…”