title: Biomechanics, diagnosis, and treatment outcome in inflammatory myopathy presenting as oropharyngeal dysphagia.
abstract: OBJECTIVE
  In patients with inflammatory myopathy and dysphagia, our aims were to determine: (1) the diagnostic utility of clinical and laboratory indicators; (2) the biomechanical properties of the pharyngo-oesophageal segment; (3) the usefulness of pharyngeal videomanometry in distinguishing neuropathic from myopathic dysphagia; and (4) clinical outcome.
  
  
  METHODS
  Clinical, laboratory, and videomanometric assessment was performed in 13 patients with myositis and dysphagia, in 17 disease controls with dysphagia (due to proven CNS disease), and in 22 healthy age matched controls. The diagnostic accuracy of creatine kinase (CPK), erythrocyte sedimentation rate, antinuclear antibody, and electromyography (EMG) were compared with the gold standard muscle biopsy. The biomechanical properties of the pharyngo-oesophageal segment were assessed by videomanometry.
  
  
  RESULTS
  Mean time from dysphagia onset to the diagnosis of myositis was 55 months (range 1-180). One third had no extrapharyngeal muscle weakness; 25% had normal CPK, and EMG was unhelpful in 28%. Compared with neurogenic controls, myositis patients had more prevalent cricopharyngeal restrictive disorders (69% v 14%; p=0.0003), reduced upper oesophageal sphincter (UOS) opening (p=0.01), and elevated hypopharyngeal intrabolus pressures (p=0.001). Videomanometric features favouring a myopathic over a neuropathic aetiology were: preserved pharyngeal swallow response, complete UOS relaxation, and normal swallow coordination. The 12 month mortality was 31%.
  
  
  CONCLUSIONS
  The notable lack of supportive clinical signs and significant false negative rates for laboratory tests contribute to the marked delay in diagnosis. The myopathic process is strongly associated with restricted sphincter opening suggesting that cricopharyngeal disruption is a useful adjunct to immunosuppressive therapy. The condition has a poor prognosis.
authors.affiliation: Department of Gastroenterology, St George Hospital, University of New South Wales, Sydney, Australia.
authors.forename: R B
authors.initials: RB
authors.lastname: Williams
authors.forename: M J
authors.initials: MJ
authors.lastname: Grehan
authors.forename: M
authors.initials: M
authors.lastname: Hersch
authors.forename: J
authors.initials: J
authors.lastname: Andre
authors.forename: I J
authors.initials: IJ
authors.lastname: Cook
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D015415:Biomarkers / Q000097:blood; D001696:Biomechanical Phenomena; D003402:Creatine Kinase / Q000097:blood; D003680:Deglutition Disorders / Q000209:etiology*; D003937:Diagnosis, Differential; D004576:Electromyography; D004947:Esophagus / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008365:Manometry / Q000379:methods; D008875:Middle Aged; D009220:Myositis / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D009422:Nervous System Diseases / Q000150:complications / Q000175:diagnosis; D010614:Pharynx / Q000503:physiopathology; D011379:Prognosis; D016896:Treatment Outcome
chemical_list: D015415:Biomarkers; D003402:Creatine Kinase