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