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Dermatomyositis

Treatment Guidelines for DM

Julie Paik, MD, MHS 
Reviewed on February 14, 2025
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Professional Guidelines

Few controlled studies are available to guide treatment strategies in dermatomyositis (DM), and treatment recommendations for DM have mostly been based on case series, case reports, and expert opinion.

Several historical classification criteria have been used for DM, including the Bohan and Peter classification (1975), Targoff criteria (1997), and the Dalakas and Hohlfeld's criteria (2003). Limitations of these systems include vague phenotypic description, unclear exclusion criteria, exclusion of autoantibodies, and exclusion of some subgroups of idiopathic inflammatory myopathies.

The more recent 2017 European League Against Rheumatism and the American College of Rheumatology (EULAR/ACR) criteria for idiopathic inflammatory myopathies have overcome some of these limitations. The EULAR/ACR IIM classification criteria offers some advantages over previously published myositis criteria, such as requiring clinically accessible non-invasive measurements.

The EULAR/ACR…

Professional Guidelines

Few controlled studies are available to guide treatment strategies in dermatomyositis (DM), and treatment recommendations for DM have mostly been based on case series, case reports, and expert opinion.

Several historical classification criteria have been used for DM, including the Bohan and Peter classification (1975), Targoff criteria (1997), and the Dalakas and Hohlfeld's criteria (2003). Limitations of these systems include vague phenotypic description, unclear exclusion criteria, exclusion of autoantibodies, and exclusion of some subgroups of idiopathic inflammatory myopathies.

The more recent 2017 European League Against Rheumatism and the American College of Rheumatology (EULAR/ACR) criteria for idiopathic inflammatory myopathies have overcome some of these limitations. The EULAR/ACR IIM classification criteria offers some advantages over previously published myositis criteria, such as requiring clinically accessible non-invasive measurements.

The EULAR/ACR guidelines employ a combination of clinical, lab, and muscle biopsy criteria.

  • Clinical criteria: Age at onset of symptoms, the pattern of muscle weakness, presence of skin manifestations and other findings (esophageal dysmotility, dysphagia).
  • Lab criteria: presence of anti–Jo-1 and elevation of muscle enzymes.

The guidelines provide a method to calculate the probability of IIM and can be used with adults and children. Patients are classified as having "possible" "probable" or “definite" idiopathic inflammatory myopathy.

The American College of Rheumatology (ACR) 2023 guidelines for screening and monitoring of ILD in patients with systemic autoimmune rheumatologic disorders, including DM recommends screening patients with both pulmonary function tests (PFTs; spirometry, lung volumes, and diffusion capacity) and high-resolution chest computed tomography (HRCT) scan at time of diagnosis. However, these recommend against screening with chest radiography, 6-minute walking test, ambulatory desaturation testing, bronchoscopy, and lung biopsy (Table 3-1).

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Management Algorithms

Treatment algorithms for DM depend on multiple factors, including presence/severity of myositis, cutaneous lesion type, extent of cutaneous involvement, cutaneous symptoms, visceral organ involvement, presence of underlying malignancy, and type of myositis-specific autoantibodies.

The typical treatment progression involves a stepwise escalation of treatment based on disease severity and response to treatment. Typically, systemic glucocorticoids are first prescribed, with tapering based on treatment response. In cases of more severe myositis, higher doses of glucocorticoids are used to slow skeletal muscle damage.

To spare use of corticosteroids, immunosuppressive agents such as methotrexate, azathioprine, mycophenolate mofetil (MMF), are prescribed with glucocorticoids at the time of diagnosis with the goal of minimizing exposure. Calcineurin inhibitors, tacrolimus and cyclosporine are typically utilized in more refractory disease especially with lung involvement With IVIG being FDA approved for dermatomyositis, it is not uncommon to use IVIG in combination to treat dermatomyositis in those not responding to 1-2 immunosuppressive agents.

During treatment, regular assessment of muscle strength, skin symptoms, and organ involvement is required. Treatment should be adjusted accordingly. Patients should be followed up at regular intervals to monitor the course of the disease and to assess response to therapy, adverse effects of therapy, systemic complications, and development of malignancy. Patients and their families should be continuously educated about the nature of DM, its progression, prognosis, and the importance of monitoring adverse effects of therapy.

References

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