Cricopharyngeal achalasia in myositis

Authors: J. Vencovský;  H. Mann
Authors‘ workplace: Revmatologický ústav, Praha
Published in: Čes. Revmatol., 16, 2008, No. 3, p. 136-139.
Category: Case Report


Dysphagia is a frequent manifestation in patients with idiopathic inflammatory myopathies. It occurs most often in inclusion body myositis but can be found in dermatomyositis and polymyositis as well. Mostly, it is linked with a worse prognosis of the disease. Aspiration of food or malnutrition can be the main complications. The most frequent cause is an acute inflammatory process or postinflammatory fibrotic changes in the area of cricopharyngeal muscle, which cause its decreased ability to relax during the act of swallowing. A short case report of a female patient with dermatomyositis with complete upper type dysphagia, which was treated by cricopharyngeal myotomy, is described.

Key words:
dermatomyositis, dysphagia, cricopharyngeal muscle


1. Williams RB, Grehan MJ, Hersch M, Andre J, Cook IJ. Biomechanics, diagnosis, and treatment outcome in inflammatory myopathy presenting as oropharyngeal dysphagia. Gut 2003; 52: 471–8.

2. Oh TH, Brumfield KA, Hoskin TL, Stolp KA, Murray JA, Bassford JR. Dysphagia in inflammatory myopathy: clinical characteristics, treatment strategies, and outcome in 62 patients. Mayo Clin Proc 2007; 82: 441–7.

3. Dietz F, Longeman JA, Sahgal V, Schmid FR. Cricopharyngeal muscle dysfunction in the differential diagnosis of dysphagia in polymyositis. Arthritis Rheum 1980; 23: 491–5.

4. Thomas FB, LeBauer S, Greenberger NJ. Polymyositis masquerading as carcinoma of the cervical esophagus. Arch Intern Med 1972; 129: 984–6.

4. Porubsky ES, Murray JP, Pratt LL. Cricopharyngeal achalasia in dermatomyositis. Arch Otolaryngol 1973; 98: 428–9.

5. Kagen LJ, Hochman RB, Strong EW. Cricopharyngeal obstruction in inflammatory myopathy (polymyositis/dermatomyositis). Report of three cases and review of the literature. Arthritis Rheum 1985; 28: 630–6.

6. De Merieux P, Verity MA, Clements PJ, et al. Esophageal abnormalities and dysphagia in polymyositis and dermatomyositis. Clinical, radiographic and pathologic features. Arthritis Rheum 1983;26:161–8.

7. Vencovský J, Řehák F, Pafko P, Jirásek A, Valešová M, Alušík Š, et al. Acute cricopharyngeal obstruction in dermatomyositis. J Rheumatol 1988; 15: 1016–18.

8. Horowitz M, Mc Neil JD, Maddern GJ, Collins PJ, Shearman DJ. Abnormalities of gastric and esophageal emptying in polymyositis and dermatomyositis. Gastroenterology 1986; 90: 434–9.

9. Jacob H, Berkowitz D, McDonald E, Bernstein LH, Beneventano T. The esophageal motility disorder of polymyositis. A prospective study. Arch Intern Med 1983; 143: 2262–4.

10. Calcaterra TC, Kadell BM, Ward PH. Dysphagia secondary to cricopharyngeal muscle dysfunction. Arch Otolaryngol 1975; 101: 726–9.

11. Berg HM, Persky MJ, Jacobs JB, Cohen NL. Cricopharyngeal myotomy: A review of surgical results in patients with cricopharyngeal achalasia of neurogenic origin. Laryngoscope 1985; 95: 1337–40.

12. Liu LW, Tarnopolsky M, Armstrong D. Injection of botulinum toxin A to the upper esophageal sphincter for oropharyngeal dysphagia in two patients with inclusion body myositis. Can J Gastroenterol 2004; 18: 397–9.

13. Bachmann G, Streppel M, Krug B, Neuen-Jacob E. Cricopharyngeal muscle hypertrophy associated with florid myositis. Dysphagia 2001; 16: 244–8.

14. Dickley BF, Myers AR. Pulmonary disease in polymyositis/dermatomyositis. Semin Arthritis Rheum 1984; 14: 60–76.

15. Manchul LA, Jin A, Pritchard KI, Tenenbaum J, Boyd NF, Lee P, et al. The frequency of malignant neoplasms in patients with polymyositis-dermatomyositis. Arch Intern Med 1985; 145: 1835–39.

16. Brigand C, Ferraro P, Martin J, Duranceau A. Risk factors in patients undergoing cricopharyngeal myotomy. Br J Surg. 2007; Aug; 94(8): 978–83.

17. Singer PA, Chikarmane A, Festoff BW, Ziegler DK. Trismus. An unusual sign in polymyositis. Arch Neurol 1985; 42: 1116–18.

Dermatology & STDs Paediatric rheumatology Rheumatology
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