Causes of Decreased Oesophageal Motility
Decreased oesophageal motility results primarily from rheumatologic diseases (especially systemic sclerosis and mixed connective tissue disease), infectious processes, eosinophilic disorders, and secondary achalasia from various inflammatory and infectious triggers. 1
Rheumatologic and Autoimmune Causes
Systemic sclerosis (SSc) and mixed connective tissue disease (MCTD) are the most significant rheumatologic causes, with particularly severe dysfunction. 1
SSc causes atrophy and fibrosis of esophageal smooth muscle, resulting in severe hypomotility and incompetent lower esophageal sphincter. 1 Esophageal motility disorders are present in 90% of SSc patients, with absent contractility being the most common finding (56% of cases). 2, 3
MCTD produces esophageal involvement resembling SSc but can affect both striated and smooth muscle (proximal and distal esophagus), with variable severity. 1
Systemic lupus erythematosus (SLE) and Sjögren's disease cause dysmotility through unclear mechanisms, with up to 80% of Sjögren's patients experiencing dysphagia. 1
Polymyositis, dermatomyositis, and inclusion body myositis affect proximal striated muscle, particularly the cricopharyngeus, with 30-80% of patients developing dysphagia. 1
Eosinophilic and Allergic Disorders
Eosinophilic esophagitis (EoE) can lead to motility disorders even after achieving histologic and endoscopic remission. 1
Eosinophils and mast cells produce neuroactive and myoactive substances that cause motility disturbances and potential neuronal destruction. 1
The relative risk of EoE in achalasia patients is 32.9 (95% CI, 24.8-42.8), particularly in those under 40 years. 1
Hypereosinophilic syndromes (HES) and eosinophilic granulomatosis with polyangiitis (EGPA) should be considered when absolute eosinophil count exceeds 1500 cells/μL. 1
Infectious Etiologies
Infectious esophagitis from Candida, HSV, and CMV can cause motility dysfunction, though more commonly in immunocompromised patients. 1, 4
Secondary Achalasia from Infections
Chagas disease causes esophageal dysfunction through immune cross-reactivity, with Trypanosoma cruzi flagellar antigen targeting the myenteric plexus, resulting in degeneration and loss of peristalsis. 1 This affects an estimated 300,000 individuals in the United States. 1
COVID-19 infection has emerged as a cause of acute-onset achalasia. 1 SARS-CoV-2 has affinity for neuronally expressed ACE2 receptors, triggering inflammation with ganglionic cell destruction. 1 Studies show 625-fold higher N protein levels in post-COVID achalasia patients compared to longstanding achalasia. 1
Inflammatory Bowel Disease
Crohn's disease can cause esophageal involvement in a minority of patients, manifesting as inflammatory, stricturing, or fistulizing changes with granulomas. 1 This typically occurs with active intestinal disease and may require biologic therapy. 1
Dermatologic Diseases
Esophageal lichen planus (ELP) causes dysmotility through mucosal involvement, presenting with narrowed esophageal caliber, multiple strictures, and pale edematous mucosa. 1 ELP can occur without skin involvement in 33% of cases. 1
Pemphigus vulgaris affects the esophagus in patients with oral disease, with 64% showing objective esophageal involvement. 1
Paraneoplastic and Malignancy-Associated
Secondary achalasia occurs with lymphoma, lung cancer, and breast cancer as paraneoplastic phenomena. 1
Risk Factors for Motility Dysfunction
Conditions predisposing to esophageal stasis increase risk of secondary motility disorders, including:
- Recent antibiotic use, systemic steroids, or immunosuppression 1
- Proton pump inhibitor use, older age, chronic alcohol use 1
- Chronic kidney disease and diabetes mellitus 1, 5
Clinical Pitfalls
Autoimmune conditions show strong association with achalasia (OR 1.49; 95% CI, 1.23-1.80), with strongest associations for SSc and Addison's disease. 1
Classic "scleroderma esophagus" (absent contractility with hypotensive EGJ) occurs in only 33% of SSc patients, emphasizing the heterogeneity of esophageal motor dysfunction in this population. 3
When evaluating new-onset achalasia, query specifically for recent COVID-19 infection, Chagas disease risk factors, and eosinophilic disease symptoms. 1