Treatment & Disease Management

Goals of therapy

Dermatomyositis requires a multidisciplinary approach involving rheumatologists, dermatologists, neurologists, pulmonologists, pathologists, physiotherapists and occupational and speech therapists.1,2

Therapy often involves a combination of pharmacological and non-pharmacological (e.g. exercise) approaches to work towards the following goals.

Goals of therapyImpact on the patient1,3
  • Limit/eliminate inflammation1
  • Restore muscle strength1,3
  • Ameliorate extramuscular manifestations (e.g. dysphagia, dyspnoea and arthralgia)3
  • Prevent damage to other organs1
  • Reduced morbidity and disability
  • Improved quality of life

Pharmacological treatment options for dermatomyositis

The goal of pharmacological treatments for dermatomyositis is to reduce inflammation, improve muscle strength and stabilise the disease.2

In absence of consensus guidelines for the treatment of idiopathic inflammatory myopathies, dermatomyositis patients are treated based on symptoms and disease severity.4

Immunosuppressive therapy is widely accepted as the mainstay of treatment for idiopathic inflammatory myopathies, with corticosteroids – commonly prednisone – widely used as first-line therapy.2,4

Other immunosuppressants are commonly used as second- or third-line therapies to avoid the serious side effects of prolonged corticosteroid use.2,4

An intravenous immunoglobulin (IVIg) has recently been approved by the US Food and Drug Administration (FDA) for treatment of dermatomyositis in adults.5

This IVIg has also been approved in Europe for use in adults with active dermatomyositis treated with immunosuppressive drugs including corticosteroids, or with intolerance or contraindications to those drugs.6

Dermatomyositis therapy aims to limit inflammation1, restore muscle strength1,3, ameliorate extramuscular manifestations3, and prevent damage to other organs.1

Suggested treatment algorithm for patients with immune-mediated myopathies4

*Disclaimer: The treatments mentioned above are not necessarily approved in all countries for use in patients with dermatomyositis. All recommendations, including dosing regimens, are based on the opinions of the authors of the cited publications. Treating physicians should always refer to the relevant package insert in their country before prescribing a product.

The degree of muscle weakness (mild or moderate/severe) helps to guide the dose of prednisone.4

In cases of moderate/severe muscle weakness, immunosuppressive therapies are usually started in parallel with corticosteroids.4

In cases of severe muscle weakness, intravenous corticosteroids are indicated.2,4

Depending on treatment response, and whether weakness is persistent or if there is other organ involvement, therapy can be escalated or de-escalated.2

Category2,4Molecule2,4Posology4EffectSide effects2
Corticosteroids
  • Oral prednisone
  • IV methylprednisolone
  • 0.75–1 mg/kg/day (Max. 60–80 mg/day)
  • IV pulse therapy 1 g/day for 3–5 days
  • Anti-inflammatory
  • Immunomodulatory
  • Weight gain
  • Osteoporosis
  • Diabetes mellitus
  • Hypertension
  • Increased risk for infections
Immunosuppressants
  • Azathioprine
  • or
  • Methotrexate
  • Start 50 mg/day; increase to 2–2.5 mg/kg/day

  • Start 10 mg/week; up to 20–25 mg/week

Immunosuppressive
  • Elevated liver enzymes
  • Gastrointestinal involvement
  • Increased risk for infections, especially in cases of severe leukopenia
  • Azathioprine Malignancy e.g. lymphoma, nonmelanoma skin cancer
  • Methotrexate Pulmonary toxicity (rare but severe side effect)
Intravenous immunoglobulin
  • Immunoglobulin
  • 2 g/kg over 5 days loading dose; 1 month later 1 g/kg monthly for 3 months
Immunomodulatory
  • Infusion reactions with headache, fever, chills
  • Allergic reactions
  • Thrombosis
  • Haemolysis
  • Haemolysis

Properties of other therapies for idiopathic inflammatory myopathies

Category2,4Molecule2,4Posology4EffectSide effects2
Immunosuppressants
  • Cyclophosphamide
  • Mycophenolate mofetil
  • IV pulse therapy every 4-6 weeks4
  • 2–3 g/day5

Immunosuppressive
  • Infections
  • Myelosuppression
  • Renal toxicity
  • Infertility
  • Secondary malignancy
  • Gastrointestinal involvement
  • Increased risk of infections, especially in case of severe leukopenia
Calcineurin inhibitors
  • Cyclosporine A
  • Tacrolimus
  • 2.5–5 mg/kg/day4
  • 0.1–0.2 mg/kg/day4
  • Inhibit T cell-mediated immune responses – suppress production of interleukin 2 (IL-2) and related cytokines
  • Evidence for a positive effect onmuscular involvementtreatment of myositis-associated interstitial lung disease
  • Hypertension
  • Nephrotoxicity
  • Hepatotoxicity
  • Malignancy
  • Inhibition of cytochrome P3A4
Monoclonal antibody anti-CD20RituximabInitially 2x 1 g followed by 1x 1 g every 6 months
  • Antibody against the CD20 antigen expressed on the surface of B cells
  • Depletion of B cells in the peripheral blood
  • Infusion reactions
  • Serious infections
  • Possible cardiotoxic effects

Pharmacological treatment options for juvenile dermatomyositis

Evidence to support treatment options in children with dermatomyositis is still lacking, and there are no approved therapies.7

Similarly to adults, the mainstay of therapy is use of corticosteroids in combination with immunosuppressive therapies.7

Adopting an early, aggressive treatment approach may prevent or stabilise organ damage and the associated disease complications such as calcinosis, the latter being associated with significant morbidity due to pain and infection risk.7

Non-pharmacological management options for dermatomyositis

ApproachBenefits
Physiotherapy and occupational therapy1
  • Improves mobility, retains motor function and prevents contractures (latter which occur particularly in juvenile dermatomyositis)
  • May help prevent side effects of corticosteroids e.g. weight gain, osteoporosis, and muscle atrophy
Physical training
  • Increases muscle strength1 e.g. with physical therapy and aerobic exercise programmes
  • Contributes to stabilisation of disease progression2
Maintenance of healthy bodyweight8
  • Reduces strain on the muscles
Improvement of diet and nutrition9
  • Anti-inflammatory effects of Mediterranean diet
  • Dietary supplements (e.g. vitamin D) is needed as dermatomyositis patients are advised to avoid sun exposure
Avoidance of UV exposure1,8,10
(e.g. use of sunscreen and appropriate cover to limit sun exposure)
  • Avoids exacerbating dermatomyositis-associated rash3
Mindfulness8
  • May reduce impact of stressful events that trigger disease flare-ups
Stopping smoking
  • Due to an association between smoking and extramuscular involvement in myositis, albeit complex and not proven to be causative11, cessation of smoking may be of benefit

References

  1. Malik A et al. Front Neurol 2016; 7:64;
  2. Glaubitz S et al. Ther Adv Musculoskelet Dis 2020; 12:1759720X19886494;
  3. Dalakas MC & Hohlfeld R. Lancet 2003; 362:971-82;
  4. Goyal NA. Continuum (MinneapMinn) 2019; 25:1564-85;
  5. Octagam® US Prescribing Information; June 2021;
  6. Octagam® SmPC; June 2021: https://www.medicines.org.uk/emc/product/4701/smpc#gref: Last accessed 18.10.2021;
  7. Enders FB et al. Ann Rheum Dis 2017; 76:329-40;
  8. John Hopkins Myositis Center. Living with dermatomyositis; https://www.hopkinsmyositis.org/educational-resources/dermatomyositis-lifestyle-options: Last accessed 19.07.2021;
  9. The Myositis Association. Diet and nutrition; https://www.myositis.org/about-myositis/treatment-disease-management/complementary-and-self-care-therapies/diet-and-nutrition/: Last accessed 18.10.2021;
  10. Oddis CV & Aggarwal R. Nat Rev Rheumatol 2018; 14:279-89;
  11. Lilleker JB et al. Ann Rheum Dis 2018; 77:30-9.

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