Case Presentation: A 72-year-old man presented with several months of progressive weakness and dysphagia with a 70-pound weight loss. He was diagnosed with rheumatoid arthritis (RA) 4 months prior based on serology and synovial biopsy and was started on hydroxychloroquine. Previous work up for weight loss and dysphagia revealed a mild stricture versus delay in relaxation at the gastroesophageal junction, multiple duodenal ulcers, and erosive esophagitis.
On admission, vital signs were normal. He was cachectic and sarcopenic. He had weakness in his upper extremities, increased tone in his lower limbs without fasciculations, and hyperreflexia demonstrated by brisk jaw jerk. There was atrophy of the intrinsic hand muscles with bilateral metacarpophalangeal and proximal interphalangeal joint tenderness and swelling.
Pharyngogram revealed severe oropharyngeal and esophageal dysphagia. Manometry showed type III achalasia. Botox injection to the lower esophageal sphincter (LES) was not efficacious. Electromyography showed generalized myopathy. Laboratory work up for infection, nutritional deficiencies, and other autoimmune processes were normal except for sedimentation rate 87 and C-reactive protein 60. A muscle biopsy demonstrated necrotizing vasculitis involving small and medium sized blood vessels.
Given the constellation of seropositive rheumatoid arthritis and muscle biopsy result, the working diagnosis was rheumatoid vasculitis.
Discussion: Hospitalists frequently encounter patients with dysphagia and constitutional symptoms, which are often attributed to acute illness or an underlying progressive process. Dysphagia is categorized as oropharyngeal or esophageal. Oropharyngeal dysphagia (difficulty initiating swallow) is commonly related to a neuromuscular process affecting the mouth, pharynx, or upper esophagus. Esophageal dysphagia (sensation of food getting stuck) is frequently caused by a structural abnormality, and work up for esophageal dysphagia typically begins with a barium swallow and/or upper endoscopy.
RA is associated with dysphagia, which is often seen in severe disease. Symptoms can be attributed to xerostomia, however, RA can also directly affect the esophagus and LES through rheumatoid vasculitis. Rheumatoid vasculitis is an extra-articular manifestation of RA that can affect the gastrointestinal tract. It is a rare complication largely due to advancements in treatment. Making a diagnosis of rheumatoid vasculitis is tedious and includes clinical findings as well as biopsy of the skin, muscle, or nerve.
Conclusions: Severe complications of RA may be overlooked. It is important to identify rheumatoid vasculitis as it is associated with high morbidity and mortality and is often resistant to treatment. As illustrated in this case, combined oropharyngeal and esophageal dysphagia associated with progressive weakness and weight loss should prompt further work up for a unifying diagnosis.