Upper Gastrointestinal (GI) Manifestations of Inflammatory Myositis: A Tale of Two Patients

Abstract

Myositis is a group of rare autoimmune disorders characterized by chronic inflammation of skeletal muscles that leads to a hallmark triad of muscle weakness, fatigue, and myalgia. Extra-muscular manifestations are sometimes seen and involve various organ systems, including the gastrointestinal (GI) tract. In this case series, two patients with polymyositis (PM) and dermatomyositis (DM), both of whom developed dysphagia as a complication of myositis, are discussed. Case 1 was a female with a known history of biopsy-proven dermatomyositis who presented with progressive peripheral edema and weakness affecting all extremities. Concurrently, she displayed symptoms of pneumonia and dysphagia associated with frequent spontaneous or self-induced vomiting to alleviate retrosternal discomfort. Esophagogastroduodenoscopy (EGD) revealed esophageal dilatation and an absence of a contractile response, consistent with myositis. Treatment comprised intravenous immunoglobulin (IVIG), mycophenolate, and lifestyle modifications, including dietary adjustments and maintaining an upright position postprandial. The second case was a female with muscle weakness and dysphagia. Video-fluoroscopic swallow assessment was significant for pharyngeal dysfunction without a sensory response to penetrated material, and the patient was at high risk of aspiration with any oral intake. The presence of pharyngeal dysfunction and dysphagia prompted treatment with IVIG, mycophenolate, and percutaneous endoscopic gastrostomy (PEG) tube placement. These cases have highlighted the upper GI complications observed in patients with myositis, accentuating the necessity for a personalized treatment approach. Timely intervention has shown promising results in symptomatic relief and improving patient outcomes. This emphasizes the importance of a multidisciplinary approach when addressing myositis-related upper GI manifestations.

Keywords: academic rheumatology; intravenous immunoglobulins (ivig); oropharyngeal dysphagia; polymyositis myalgia; s: dermatomyositis.

Figure 1. XR esophagogram of patient 1

The green arrow shows dilatation of the distal esophagus and mild narrowing of the gastroesophageal junction. Characteristics of achalasia.

Figure 2. CT of chest without contrast

Green arrow A: small amount of debris in distal esophagus; Green arrow B: airspace consolidation with groundglass opacities.

Figure 3. Video-fluoroscopic study

Green arrow: shows penetration without aspiration drinking thin liquid.