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[3 min read] Update on treatments and prognosis of dermatomyositis

Dermatomyositis is an autoimmune disease affecting both skin and muscle. Oral steroids are the first-line treatment, but no consensus exists regarding dosing, length of treatment, tapering speed, or when to add which immunosuppressant in case of steroid resistance.

Randomised-controlled trials are scarce and most are retrospective, and there is a particular lack of data on long-term therapies to aid decisions on the most effective long-term treatment.

Empiric evidence suggests that with an initially aggressive treatment with oral steroids followed by a slow taper, during which disease activity is adequately controlled, patients can be off therapy within typically 24–48 months, and remain disease-free without medication for over 20 years.

Biologics such as rituximab have shown good results in the treatment of refractory dermatomyositis. New, targeted therapies have been reported to improve the disease in single cases or case series, but have to be evaluated for efficacy in randomised controlled trials.

However, it is worth noting that many studies on treatments for dermatomyositis assess outcomes of the muscle disease, but the response of the skin may not be the same; skin disease often does not respond as well. Another limitation of these studies is that they often lump together patients with dermatomyositis and polymyositis, as well as other myopathies.

Other treatments for patients with dermatomyositis include intravenous immunoglobulin and plasma exchange, as well as immunosuppressive agents such as plaquenil (hydroxychloroquine) and rituximab, which is the best studied and has been shown to have a steroid-sparing effect. In a small case series, rituximab had a benefit of 75 to 86 per cent, but it doesn’t work as well as it does in pemphigus. Further, TNF inhibitors have shown differing benefits, but are overall not worthwhile.

Read more recent research.

Source: Verena Isak & Joseph L. Jorizzo (2018) Recent developments on treatment strategies and the prognosis of dermatomyositis: a review, Journal of Dermatological Treatment, 29:5, 450-459, DOI: 10.1080/09546634.2017.1403549

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