Background/Purpose: Polymyositis (PM) and dermatomyositis (DM) are autoimmune inflammatory diseases characterized by proximal myositis. Dysphagia has been reported to develop in 35 to 62% of PM/DM patients and known as poor prognosis factor. This study aimed to determine the clinical feature of PM/DM patients presenting severe deglutition disorder.

Methods: Consecutive patients with PM/DM who visited National Hospital Organization Tokyo Medical Center between April 2010 and March 2019 are included in this study. We compared the following clinical features between the patients with and without severe dysphagia: age of onset, maximum levels of serum creatine kinase (CK), sense of dysphagia, manual muscle test (MMT) score, and complication of malignancy. Severe dysphagia was defined as having difficulty swallowing which needed gastrostomy.

Results: A total of 73 patients with PM/DM were identified. Among them, 5 patients developed severe dysphagia. Patients with severe dysphagia presented older age of onset (mean age 69.4 ± 12.0 vs 51.8 ± 15.7, p = 0.016), more frequent muscle weakness (MMT grade ≦ 4)(100% vs 56.6%, p = 0.048）, more frequent complication of malignancy (80.0% vs 11.8%, p= 0.0005) and sense of dysphagia (100% vs 10.7%, p< 0.0001). On the other hand, there were no significant differences in maximum serum CK levels between the patients with and without severe dysphagia (21884.0 ± 10275 U/L vs 5544.5 ± 3043 U/L, p = 0.1788).

Conclusion: These results indicate that severe dysphagia develops frequently in PM/DM patients with older onset, muscle weakness, complication of malignancy and sense of dysphagia. When any of these clinical features are noticed, it is necessary to consider the risk of severe dysphagia.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-features-of-polymyositis-and-dermatomyositis-patients-with-severe-dysphagia/