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Myositis Flare vs Steroid Myopathy — SCE Rheumatology MCQ

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ModerateMyositisMyositis Flare vs Steroid MyopathySCE Rheumatology

A 55-year-old woman with dermatomyositis has been stable on prednisolone 5 mg daily and azathioprine. Over 2 weeks she develops worsening symmetrical proximal weakness and recurrent erythematous papules over the extensor surfaces of the metacarpophalangeal and interphalangeal joints. Her creatine kinase has risen from 200 to 4500 U/L. Medication review and initial assessment reveal no infection or alternative myotoxic drug exposure. What is the most appropriate next step?

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Correct answer: D — Manage as an active dermatomyositis flare and promptly intensify glucocorticoid and immunosuppressive treatment

The marked CK rise, recurrent symmetrical proximal weakness and return of Gottron papules indicate an active dermatomyositis flare. She therefore requires prompt escalation of glucocorticoid-based treatment and review or intensification of steroid-sparing immunosuppression. Glucocorticoid myopathy can cause painless proximal weakness, but it is non-inflammatory, usually develops insidiously and typically has a normal CK; it cannot explain the recurrent characteristic rash. Azathioprine is used as a steroid-sparing treatment for myositis and is not the likely cause of this clinicobiochemical pattern. EMG or muscle MRI may help when disease activity is uncertain, but delaying treatment for confirmatory testing is inappropriate here. Thyroid dysfunction remains a differential for weakness and CK elevation but would not account for the recurrent Gottron papules.

Reference: Oldroyd AGS et al. British Society for Rheumatology guideline on management of paediatric, adolescent and adult patients with idiopathic inflammatory myopathy. Rheumatology. 2022;61:1760–1768. https://www.rheumatology.org.uk/guidelines