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Dermatomyositis treatment objectives: Reducing symptoms and minimize extra-muscular involvement, enhancing muscle strength, and managing inflammation. All while avoiding side effects.

First-line therapy: corticosteroids
Extremely successful in lowering immunological response and decreasing inflammation. For instance, high-dose daily prednisone (0.7 to 1mg/kg/day, up to a maximum of 60 mg daily).

Alternatives according to patient type:

·      Severely weak patients: 3-5dayIV methylprednisolone before starting oral prednisone

·      Increased risk of steroid-induced side effects: High-dose prednisone and second-line agent immediately to taper prednisone as soon as possible

·      Mild disease patient: Low-dose prednisone with gradual increase until response

Steroid-sparing immunosuppressive agents

These impede the production of DNA, which impacts the growth of immune cells. Allow the use of glucocorticoids at a reduced dosage while preserving disease control. therefore reducing the negative effects of long-term steroid use (examples methotrexate and azathioprine).

Treatment Protocol

Initial Phase: High doses of glucocorticoids to rapidly reduce inflammation.

Continue without taper until strength normalizes (2 to 4 months)
Taper Phase: The dosage of glucocorticoids is gradually reduced as the patient's symptoms get better. Tapering to drastically may lead to relapse

Recommended taper

·      10 mg every 4 weeks until 20 mg/day

·      5 mg every 4 weeks until 10 mg/day

·      1-2.5 mg every 4 weeks thereafter

Long term control: Patients undergo routine monitoring to determine the efficacy. Most patients will require an ongoing small dose of prednisone.

Second-line medication:

Add second-line medication if

·      incomplete response to high-dose prednisone after 2-4 months

·      patient relapses during prednisone treatment

Start at disease onset with second-line agent if

·      Severe weakness at onset

·      Multisystem involvement

·      Contraindications to corticosteroids

NOTE:
Ineffective treatment: Reconsider diagnosis (differentials: muscular dystrophy)

REFERENCE
Lundberg, I. E., de Visser, M., & Werth, V. P. (2018). Autoimmune myopathies: Updates on evaluation and treatment. Neurotherapeutics, 15(4), 976-994. https://doi.org/10.1007/s13311-018-00676-2

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A 61-year-old Vietnamese woman diagnosed with dermatomyositis (DM), presenting with symptoms such as Gottron’s papules, heliotrope rash, cutaneous ulcers, generalized weakness, pain, and significant weight loss, but normal creatine kinase (CK) levels. She exhibited signs of interstitial lung disease and tested positive for anti-MDA5 and anti-SAE antibodies, indicative of clinically amyopathic dermatomyositis. Despite receiving standard DM treatment (oral prednisolone, hydroxychloroquine, mycophenolate, and topical betamethasone), which improved her skin symptoms, she remained weak and unable to perform daily activities.

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A 44 year old female patient living in Purano-baneshwor, Kathmandu, Nepal with hyperpigmented erythematous rash all over her body along with V-shaped rashes on the neckline since 2 months. Initial symptoms as mentioned by the patient included itchy rashes and photosensitivity. No history of headache, fever, and weight loss with a normal vital test. In addition, she had no surgical as well as medical history of chronic disease. She had progressed symptoms like muscle pain (especially in the left arm and thigh), unable to walk upstairs, inability to stretch hands, unable to turn neck, and difficulty in swallowing food.