Date: Monday, November 8, 2021
Session Type: Poster Session C
Session Time: 8:30AM-10:30AM
Background/Purpose: Dermatomyositis (DM) and polymyositis (PM) are rare heterogenous systemic autoimmune disorders of the skin, muscles, and other organs that may have a devastating impact on patients and care partners life. The objective of this study was to systematically review and synthesize evidence on clinical and humanistic burden and unmet needs in the management of DM/PM.
Methods: A systematic literature review (SLR) was conducted in MEDLINE and Embase databases to identify studies in children and adults with DM/PM, published in the English language between 2011–2021. Only primary studies of any design enrolling 10 or more patients were included, irrespective of country or region. The current abstract summarizes preliminary SLR results on the clinical and humanistic burden of DM/PM in the United States (US).
Results: A total of 3624 records were retrieved from medical databases, 393 records underwent full-text review, and 210 were included in data abstraction. An additional 8 papers were included from searching reference lists of identified studies. There were 32 US studies that reported on the natural history of the disease and/or comorbidities (n=27) and quality of life (QoL) of patients or caregivers (n=8). There were 24 retrospective and 8 prospective studies, with a sample size ranging from 17 to more than 160,000 patients. Patients with DM/PM had an increased risk of comorbidities, including various types of infections (19–42%), malignancies (6–17%) and cardiovascular-related disorders (1–20%), compared to unmatched or matched non-DM/PM controls. Concerns about malignancy risk contributed to increased depression and anxiety. Remission occurred in only 33–38% of adult DM patients. About 26–50% of patients with juvenile DM/PM (JDM/JPM) often had a chronic course of disease characterized by persistently active disease, and a 36% rate of active disease 10-years post-diagnosis. Diseases flares were reported by 73% of patients within the past year and their increased frequency trended with a longer mean duration of illness (p< 0.001). Disease flares significantly contributed to poorer QoL due to increased disability and pain (p< 0.001). Adult patients with DM, compared to other dermatologic disorders such as cutaneous T-cell lymphoma and non-melanoma skin cancer/actinic keratoses, had a significantly worse Skindex-29 emotional subscore, worse SF-36 role-emotional, physical, and social functioning subscore, and greater fatigue compared to healthy controls (p< 0.05). DM had a detrimental impact on QoL which correlated with skin disease activity. JDM affected the entire family with difficulties in family functioning, communication problems, sibling distress, and increased number of conflicts. Parents of patients with JDM reported higher levels of worry, anger, depression, and reduced physical functioning, than parents of children with chronic diseases.
Conclusion: DM/PM are associated with multiple comorbidities affecting multiple QoL domains, especially in the physical and social/emotional realm. Despite various therapies available, a notable proportion of patients seem not to achieve sustainable remission indicating a high residual unmet need.
To cite this abstract in AMA style:Venuturupalli S, Goyal N, Fortune N, Leff R, Pisarczyk K, Aleksanderek I, Palaniswamy K, Henig N. Clinical and Humanistic Burden of Dermatomyositis and Polymyositis in the United States: A Systematic Literature Review [abstract]. Arthritis Rheumatol. 2021; 73 (suppl 9). https://acrabstracts.org/abstract/clinical-and-humanistic-burden-of-dermatomyositis-and-polymyositis-in-the-united-states-a-systematic-literature-review/. Accessed January 30, 2023.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-and-humanistic-burden-of-dermatomyositis-and-polymyositis-in-the-united-states-a-systematic-literature-review/