Abstract
BackgroundDisease characteristics of classic dermatomyositis (DM) and clinically amyopathic DM (CADM) are well-established, but there exists limited knowledge on disease progression of these subtypes. ObjectiveTo longitudinally track and characterize classic DM and CADM patients that experience changes in disease presentation. MethodsWe conducted a retrospective review of prospectively collected data on 269 DM patients of a longitudinal database. ResultsA total of 51% had classic DM and 49% had CADM. Forty percent of the classic DM patients became post-myopathic (PmDM). Median CDASI-A score was lower in PmDM patients than classic DM patients (13.0 vs 16.0), but 45% of the PmDM patients had CDASI-A scores >14. Five percent of the CADM patients developed muscle involvement. Compared to CADM patients, those that developed muscle symptoms had milder skin disease before subtype conversion (median CDASI-A 12.0 vs 16.0) and at subtype conversion (median CDASI-A 9.0 vs 16.0). LimitationsRetrospective study conducted at a single tertiary-care dermatology clinic. Conclusion40% of classic DM patients became PmDM. The majority continue with muscle disease, and many continue to have moderate/severe skin disease. CADM has a low risk of progressing to muscle disease, with extent of skin disease as a potential predictive factor.
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