Treatment options from bench to bedside for adult dermatomyositis

ABSTRACT

Introduction: Current literature has minimal solid data to guide therapeutic intervention in adult dermatomyositis and studies that include systemic inflammatory mechanism suppression, musculoskeletal and extra-muscular tissue damage prevention, and comorbidities.

Area covered: This systematic literature review discusses drug and non-drug treatments in patients with adult dermatomyositis. The review includes search results on the Medline (PubMed), Cochrane, and Embase databases and lonely allows available research studies published before October 2019.

Expert opinion: The importance of non-drug treatments in patients with dermatomyositis has been highlighted, especially the multidisciplinary approach of minimizing damage and paying special attention to multiple possible organ and system involvement (mainly the gastrointestinal, cardiac, lung, joint, and cutaneous impairments) and comorbidities. Considering the limitations caused by muscle involvement, special attention should be given to the establishment of early exercise programs. Concerning drug treatments, glucocorticoids with intravenous human immunoglobulin could be the first-line treatment for refractory cases and/or cases where immunosuppressive/immunomodulatory drugs are contraindicated. Among immunobiological drugs, rituximab has the most evidence in the literature to support its use.

KEYWORDS:

• Dermatomyositis
• drugs
• idiopathic inflammatory myopathies
• systemic autoimmune myopathies
• treatment

Article highlights

• Education for patients, as well as their families and/or caregivers, is of significant importance to improving their quality of life

• Multidisciplinary treatments should be considered to minimize the correlated risk for disease-related morbidity with the possibility of multisystemic involvement. We highlight early diagnoses and special attention to psychiatric, gynecological, and metabolic syndrome disorders. The inclusion of a physical exercise program seems to be beneficial and safe for adult DM patients and should be used as an addition to pharmacological therapies in all stages of DM

• Glucocorticoid are the first-line agents for treating in DM; IVIg is considered for refractory situations or when immunosuppressive/immunomodulatory agents are contraindicated

• Consideration should be given to the early introduction of immunosuppressive drugs, especially azathioprine, methotrexate, and cyclosporine

• Regarding immunobiologicals, the greatest evidence refers to rituximab for individuals who do not respond to immunosuppressive/immunomodulatory agents. Also anti-TNF drugs are not recommended

• In specific cases, one must consider the severity of the manifestations. Treatment can involve methylprednisolone pulse therapy, alone or associated with IVIg, with early introduction of immunosuppressant drugs and rituximab. In the case of associated neoplasms, concomitant treatment of malignancy is imperative

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Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was funded by Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq) #303379/2018-9 to SKS, and Faculdade de Medicina da USP - SP to SKS.