Rituximab Therapy in Polymyositis-associated Severe Dysphagia: A Case Report

Abstract

Polymyositis is one of the inflammatory myopathies. It is a chronic autoimmune disease that usually involves the proximal muscles. It is caused by an inflammatory infiltrate of the skeletal muscle. Notably, dysphagia occurs in one-third of the patients. Traditional treatment with steroids and immune modulators has been reported to have varied outcomes and recurrent symptomatic flares. The dysphagia which occurs in such myopathies may be one of the recurrent and severe symptoms of myopathies, often associated with considerable mortality and morbidity. Dysphagia, an often-noted clinical presentation of polymyositis (PM) /dermatomyositis (DM) can be the initial sign of idiopathic inflammatory myopathies. It can cause considerable morbidity in patients such as reduced pharyngeal contractility, hypomotility of the esophagus, cricopharyngeal dysfunction, and decreased laryngeal elevation. Here is a case of severe dysphagia in a patient with muscle and skin involvement in PM. Steroids are the first line of treatment for patients with myositis and the same was adopted initially for the current case. Rituximab was used after unsuccessful attempts with other recommended first-line treatments. Reversal of dysphagia and overall improvement were achieved with the use of rituximab and intravenous immunoglobulins. The patient demonstrated significant improvement in swallowing, with reasonable improvement, following which the percutaneous endoscopic gastrostomy (PEG) tube was removed. After 15 months, the patient showed 100% resolution of all symptoms and was advised to stop all medications. Therefore, we suggest that this combination may be considered in cases with dysphagia flares in DM, where traditional treatment options have shown no benefit.