ReportA systematic review of adult-onset clinically amyopathic dermatomyositis (dermatomyositis siné myositis): A missing link within the spectrum of the idiopathic inflammatory myopathies
Section snippets
Clinical nomenclature employed
The underlying hypothesis behind our work in this area has been that the failure to distinguish between clinical subphenotypes dominated by the cutaneous manifestations of DM has hindered the optimal management of such patients with respect to diagnosis, treatment, and prognosis. This hypothesis is especially true for patients exhibiting the hallmark cutaneous manifestations of DM for prolonged periods of time without developing clinically significant muscle weakness. Laboratory testing alone
Epidemiology/demographics
A total of 301 adult-onset cases meeting either our primary or secondary case definitions were identified in the world literature. Among these, 291 were best classified as CADM and 10 as PRMDM. In the CADM group, the average age was 50 years with 73% being female. The average duration of skin disease was 3.74 years with a range from 6 months (by definition) to greater than 20 years. That is to say, on average these 291 CADM patients had experienced DM skin disease for 3.74 years without ever
Discussion
The goal of this review was to provide a more-comprehensive perspective on the clinical and laboratory features and prognosis of adult-onset CADM patients. Accordingly, we have summarized data from all reported cases of adult-onset CADM that our case ascertainment strategy identified up to May 1, 2004. We identified 291 reported cases of CADM and 10 of PRMDM from 20 nations representing 7 races/ethnicities, which establishes that the CADM subphenotype, like CDM, is seen throughout the world. We
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Funding sources: Dr Sontheimer's contributions to this project were supported by endowment funding from the Department of Dermatology at the University of Iowa Hospitals and Clinics (The John S. Strauss Endowed Chair in Dermatology) and the Department of Dermatology at the University of Oklahoma Health Sciences Center (The Richard and Adeline Fleischaker Chair in Dermatology Research). No other source of funding was associated with this study.
Conflict of interest: The authors certify that any affiliations with or involvement (eg, honoraria; education grants; speakers' bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements) in any organization or entity with any financial interest in the subject matter or materials discussed in this manuscript are disclosed on the paragraph immediately above.