Review
The treatment and prognosis of dermatomyositis: An updated review

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Dermatomyositis (DM) is an idiopathic inflammatory myopathy. The mainstay of treatment for DM is oral corticosteroids. However, the dose and length of treatment is debated. Adding to the confusion, there have been no randomized controlled studies comparing the use of various corticosteroid doses and taper rates, and no controlled long-term studies assessing the hypothesis that, unlike systemic lupus erythematous, patients with DM can often achieve long-term remission off therapy. This literature review supports an approach that prednisone should be started at about 1 mg/kg/d, which is then tapered slowly based on the response. As patients respond differently to prednisone, additional therapies may be necessary. When to initiate these therapies requires clinical judgment. In addition, as we learn more about the pathophysiology of DM, newer medications that target specific mechanisms in the immune response may help us better treat the disease. Evidence-based data with long-term follow-up will allow for selection of the best treatment to maximize long-term remission, not simply short-term lowering of the systemic corticosteroid dose.

Section snippets

Corticosteroids

Adults with DM who have active, confirmed muscle disease are generally given systemic corticosteroids. High-dose corticosteroids (>0.5 mg/kg/d) are the mainstay of treatment for this disease. The American Academy of Dermatology (AAD) recommends systemic corticosteroids for initial control of muscle disease and, in most instances, a sufficient initial dose should be 0.5 to 1.5 mg/kg/d. After the muscle disease is controlled, the dose can be tapered slowly.7 The AAD did not address specific taper

Dermatologic manifestations

In DM, the dermatologic manifestations are often difficult to treat. They may have a discordant response to therapy from the muscle disease, and can continue to be refractory to treatment after muscle weakness has resolved.32 To treat skin manifestations, the AAD7 recommends aggressive sun protection with physical barriers and sunscreen, and topical corticosteroids.

Hydroxychloroquine has been used in treating DM, which is endorsed by the AAD.7 Woo et al128 treated 7 patients with variations of

Prognosis

As treatments for DM have changed, so has the prognosis. It is important to identify prognostic factors, as these may eventually help improve treatments for patients with specific characteristics of DM. Mortality has varied from 8.9% to 52%,24, 51, 148, 149, 150 however, the large range may be a result of disease variation across patients. The disease subtypes Bohan and Peter4 identified are frequently analyzed together. Duration of follow-up can also affect the perceived prognosis. One-year

Our approach to the treatment of patients with DM

Our view is that the majority of patients with DM can experience long-lasting remissions (>20 years follow-up in many patients) off therapy if the treatment of the DM is initially aggressive, and then is gradually tapered over 2 to 3 years without allowing muscle disease (as assessed by muscle enzymes, clinical evaluation of proximal extensor muscle strength, and other standard measurements) to flare (ie, flares are rapidly and completely suppressed). The dermatologic disease, unfortunately for

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